Dermatomyositis

Myositis is a group of inflammatory myopathies that are most commonly thought to impact the muscles (which they do) but they can also affect the heart, lungs, GI tract, and be associated with the development of cancer. Myositis is considered autoimmune in nature; the body begins attacking its own muscles, connective tissue, skin, blood vessels and other organs. It is estimated that between 50-75,000 people in the U.S. have one of the forms of myositis. This makes it among the rare diseases. As such, there are no dedicated treatments or therapies that have been developed specifically for any of the inflammatory myopathies. Broadly, there are three main types of myositis: dermatomyositis, polymyositis, and sporadic inclusion body myositis. Typically, the easiest to diagnose because of its hallmark, purplish, skin rash, is dermatomyositis. The rash that accompanies dermatomyositis most often forms on the face, upper back, chest, elbows, knees and knuckles. It is more typical for muscle weakness to come later in the progression of dermatomyositis. After the disease process starts to take hold, vasculitis, or swelling of the blood vessels, begins to cause the muscles closest to the core of the body to become weakened. It is most common for muscle weakness to be symmetrical and progressive. As with polymyositis, the kinds of drugs used to treat dermatomyositis are typically immunosuppressive and anti-inflammatory drugs. Often, high-dose prednisone is used to try and halt the disease’s rapid progression, while drugs like azathioprine, mycophenolate, and methotrexate might be used for long-term treatment. Sporadic inclusion body myositis (sIBM) is the most common of the inflammatory myopathies and generally impacts individuals after the age of 50; because of its slow progression, experts aren’t actually sure that’s when the disease typically starts, but it is most commonly diagnosed after that age. Unlike other forms of myositis that may be relapsing/remitting in nature, sIBM is progressive — the body is continuing to atrophy once the illness has been acquired. This form is distinct in a couple of other ways too. Unlike other forms of myositis, the weakness in sIBM is not necessarily symmetrical in its presentation. Also, rather than wasting that begins with the proximal, or core muscles, sIBM often begins with distal muscles like the feet, hands, ankles and lower legs. Having difficulty with falls, balance, or drop-foot are common complaints at the onset of sIBM. The symptoms of sIBM and rate of progression varies significantly from person to person and can speed up or slow down over time with little rhyme or reason. Because of its many faces and how difficult it is to diagnose, patients with sIBM often go undiagnosed for many years. Their diseases are also often misdiagnosed as the effects of aging, as multiple sclerosis, or as polymyositis. Polymyositis, like other myotises, is often referred to as a “muscle wasting” disease. Inflammatory cells work into the fibers of smooth (skeletal) muscle tissue and burst the muscles, dissolving them right off the bone. It can be slow or rapid in its progression and can be more difficult to identify because it does not have any external markers (like dermatomyositis’ rash) to make it evident. It is most common for polymyositis to begin, like dermatomyositis, with the core or proximal muscles being symmetrically impacted. I have myositis, and my complaints began with things like, “I’m having trouble rising from a seated position.” I started needing assistance getting off the couch or toilet. Then it progressed to I can’t lift my arms above my head to wash my hair, and eventually the inability to roll over in bed without assistance and having difficulty swallowing. Unfortunately, once muscle fibers are destroyed, they cannot be recreated. Therefore, the rehabilitation after the onset or relapse of the condition (both poly and dermatomyositis are relapsing/remitting conditions) is arduous and contingent upon beefing up what muscle remains. Like dermatomyositis, the drugs used to treat polymyositis are typically immunosuppressive and akin to the drugs given to RA patients. Rheumatologists specialize in the treatment of rheumatologic or autoimmune diseases. Therefore, it is typical for those of us with myositis to have a rheumatologist (if we are lucky) to monitor our condition and treatment. However, I live in a city with a population of approximately 175,000 people and see a very competent rheumatologist and I am his only myositis patient. In addition to seeing a rheumatologist, because of the way these diseases impact multiple systems, it might also be necessary to have a neurologist, dermatologist, and/or cardiologist consult about aspects of the condition. While these diseases are said to not have any genetic link, the only other person I have known with polymyositis is my mother. So… That leads me to believe they don’t have enough evidence to understand what the genetic links of these diseases may be. What is also true is that those of us who have one autoimmune condition are at great risk for having multiple autoimmune conditions. Fun. May is Myositis Awareness Month. Thanks for hanging in here and educating yourself about this rare disease. If you are seeking resources for yourself or a loved one about these muscle-related conditions, you can get more information, you can find connections, and learn from experts at Myositis.org and UnderstandingMyositis.org.

I don’t work due to my cluster of diseases, at the forefront being dermatomyositis, my body riddled with weakness, rashes, fatigue and pain. I’ve been immune suppressed for much of the past 26 years. I know my pandemic experience was somewhat unique. My husband was fortunate to be able to work at home for the past 53 weeks and four days. I immediately started sewing masks, free for friends and family, when we shut down. I stopped counting at 300 masks. Our wonderful son and his partner brought us our groceries; we had everything else delivered. After the initial quarantine we ventured out in public a little, in the summer lull, but quickly retreated as the numbers of cases started to climb. Since then, we avoided indoor public spaces with two exceptions, my doctor appointments, and our daughter’s practices at the ice rink. Even those were tenuous, I had virtual appointments when possible and she only skated on sessions with fewer than five people for the majority of the pandemic. She periodically completely stopped practices for weeks at a time as the numbers of cases escalated. Family members visited with us outside, no matter the weather, masked, distanced, joyously, unabashedly and unreservedly grateful to be together however we could…all to keep me from getting COVID-19. My family did everything they could to keep me safe, and I am infinitely grateful. I was privileged to be wrapped in a cocoon of love, masks, cleansing wipes and sanitizer. If you want to understand love and compassion, look into the eyes of your 4-year-old nephew as he puts his mask on because, “Hold on, Auntie. I hate ‘rona, I need to get my mask on, so I won’t make you sick.” There was no fear in that statement, only a declaration of pure love and protection. It showed the understanding of the situation we were living in, and his role as a loving family member protecting someone from the outside world. We (by “we” I mean my unflappable husband and daughter) spent months wiping down everything as it came into the house. Then as the scientists learned more about transmission types and prevalence we relaxed. We still use sanitizer like water. We still wipe our faces and arms with baby wipes when we leave public places, although now that I am fully vaccinated I am feeling a little less nervous about every surface I touch. Which brings me to coming out of my cocoon… This week, after 53 weeks, my daughter traveled to skate at a training facility in another state for a week of training and my husband went back to the office for the first time. It feels really abrupt and I have to admit I’m nervous. I think I will miss the relative safety of the cocoon my family built around me. Although I guess looking back over the past couple of weeks it really isn’t so abrupt. They made “an emergency” road trip last week to get her new skates when hers broke down suddenly. I picked up take-out by myself. I walked into a couple of stores. I hugged my little nephews. I ate dinner with my friend who is also fully vaccinated. All things that felt impossible and unreachable a couple of weeks ago. This morning as my husband and I ate breakfast together before he left for work, while we texted with our daughter as she woke up to get ready for her first day of training at this new facility, I was suddenly overwhelmed with the feeling that my cocoon of safety was gone. I had a rush of feelings: gratitude, fear, anxiety, sadness, excitement and shock that we are moving forward. A cold puts me out for weeks. A sinus infection gives me “walking” pneumonia. I’m participating in the Johns Hopkins study to monitor antibodies in vaccinated chronic illness patients and when I got the email stating that my body had made antibodies in response to my first shot I crumbled into tears of joy and liberation. A part of me never believed, even with our precautions, that I would survive this pandemic; the knowledge that my body was trying to mount a defense, no matter the size, is encouraging and gave me great relief. I am happy and excited to be moving forward. I mourn deeply for those we have lost and sincerely hope we, as a people, can learn lessons from this pandemic about how to treat each other; but I am worried that many will go back to normal as if it never happened. I know my experience of being safely cocooned is not that same for everyone, but I imagine others with chronic illness and with immune suppression will experience some level of processing of emotions as they and their loved ones re-enter the world as well. I hope by telling my story it helps to normalize that it is OK to be scared, to even miss the time of being “stuck” at home, to whatever extent you were. I know that even through all the anxiety, pain, sadness and anger I felt, for the past year, I felt an immense amount of gratitude for so many things and emerging is not easy. We can only control the things we have control over, so we will continue to mask (double mask in crowds) and do what we can to stay safe, but it is nice not to be so afraid anymore.