Diagnosis (?)
Words that have many syllables tend to be bad things. Undemonstrativeness. Compartmentalization. Unilateralism. Abominability. Remilitarization. Misidentification. Unmanageability. Oversimplification. Irresponsibility.
And this new one that I have just learned: Dermatomyositis.
Dermatomyositis is a rare autoimmune disease marked by muscle weakness and a distinctive skin rash. People who have dermatomyositis also sometimes feel tired and run down. In adults, dermatomyositis usually occurs from the late 40s to early 60s and affects more females than males. There's no cure, but periods of symptom improvement (remission) can occur.
After dozens of negative blood tests have ruled out other diseases from Lyme Disease to lupus, amyopathic dermatomyositis is my "working diagnosis", according to my rheumatologist, Dr. Alan Gorn. My illness this spring does not perfectly correspond to the typical symptoms - I had no muscle weakness and only a mild rash - but I tested positive for autoantibodies against melanoma differentiation-associated protein (MDA5), which is a hallmark of the disease.
Interestingly, DM in adults has been linked to an increased likelihood of developing cancer, particularly of the cervix, lungs, pancreas, breasts, ovaries, and gastrointestinal tract. It has been identified as a precursor to as well as a result of breast cancer - and, frighteningly, a harbinger of recurrence. However, because I have been imaged and scanned and biopsied to the nth degree with no sign of cancer, Dr. Gorn said he is not concerned about malignancy in my case.
For now, I have started a daily dose of hydroxychloroquine (Plaquenil), which is considered a disease-modifying anti-rheumatic drug (DMARD). Hydroxychloroquine was originally created to treat malaria, but today it is used to treat rheumatoid arthritis, lupus, and other autoimmune diseases. It is not clear why it is effective at treating autoimmune diseases, but it is hypothesized that it interferes with the communication of cells in the immune system. In my case, Dr. Gorn believes that it will stave off flares and potentially make them milder. If I do become symptomatic again, there are more powerful medications that we may try, including corticosteroids like prednisone, immunosuppressive/cytotoxic drugs like methotrexate, or a monoclonal antibody called Rituxin. These are fairly nasty drugs that I would really prefer not to have in my body, but it is good to know that they are out there if things get bad.
The other measure that Dr. Gorn really stressed is avoiding exposure to sun, the most common trigger of skin symptoms. Many people with DM are photosensitive, meaning they are prone to developing a rash, or experience a worsening of a rash, when exposed to ultraviolet rays. The reasons UV light triggers dermatomyositis are not well understood, but it may exacerbate the inflammation that leads to rashes. It's been a long time since I purposely sought out the sun, but in LA it's hard to avoid completely. However, being more vigilant about wearing sunscreen and a hat is not difficult, and something I'd be better off doing in any case.
When I think about it a little harder, I find that there are actually some good many-syllabled words. Inalienable. Environmentalism. Collaborativeness. Individuality. Spiritualization. Imaginativeness. Indefatigableness. Indestructibility. Appreciativeness.
And this one: Foreseeability. This is what I get from a working diagnosis - not the answer, not a cure, but insight into what the future could hold, and some assurance that my doctors will know what to do if my working diagnosis becomes real.
And this new one that I have just learned: Dermatomyositis.
Dermatomyositis is a rare autoimmune disease marked by muscle weakness and a distinctive skin rash. People who have dermatomyositis also sometimes feel tired and run down. In adults, dermatomyositis usually occurs from the late 40s to early 60s and affects more females than males. There's no cure, but periods of symptom improvement (remission) can occur.
After dozens of negative blood tests have ruled out other diseases from Lyme Disease to lupus, amyopathic dermatomyositis is my "working diagnosis", according to my rheumatologist, Dr. Alan Gorn. My illness this spring does not perfectly correspond to the typical symptoms - I had no muscle weakness and only a mild rash - but I tested positive for autoantibodies against melanoma differentiation-associated protein (MDA5), which is a hallmark of the disease.
Interestingly, DM in adults has been linked to an increased likelihood of developing cancer, particularly of the cervix, lungs, pancreas, breasts, ovaries, and gastrointestinal tract. It has been identified as a precursor to as well as a result of breast cancer - and, frighteningly, a harbinger of recurrence. However, because I have been imaged and scanned and biopsied to the nth degree with no sign of cancer, Dr. Gorn said he is not concerned about malignancy in my case.
For now, I have started a daily dose of hydroxychloroquine (Plaquenil), which is considered a disease-modifying anti-rheumatic drug (DMARD). Hydroxychloroquine was originally created to treat malaria, but today it is used to treat rheumatoid arthritis, lupus, and other autoimmune diseases. It is not clear why it is effective at treating autoimmune diseases, but it is hypothesized that it interferes with the communication of cells in the immune system. In my case, Dr. Gorn believes that it will stave off flares and potentially make them milder. If I do become symptomatic again, there are more powerful medications that we may try, including corticosteroids like prednisone, immunosuppressive/cytotoxic drugs like methotrexate, or a monoclonal antibody called Rituxin. These are fairly nasty drugs that I would really prefer not to have in my body, but it is good to know that they are out there if things get bad.
The other measure that Dr. Gorn really stressed is avoiding exposure to sun, the most common trigger of skin symptoms. Many people with DM are photosensitive, meaning they are prone to developing a rash, or experience a worsening of a rash, when exposed to ultraviolet rays. The reasons UV light triggers dermatomyositis are not well understood, but it may exacerbate the inflammation that leads to rashes. It's been a long time since I purposely sought out the sun, but in LA it's hard to avoid completely. However, being more vigilant about wearing sunscreen and a hat is not difficult, and something I'd be better off doing in any case.
When I think about it a little harder, I find that there are actually some good many-syllabled words. Inalienable. Environmentalism. Collaborativeness. Individuality. Spiritualization. Imaginativeness. Indefatigableness. Indestructibility. Appreciativeness.
And this one: Foreseeability. This is what I get from a working diagnosis - not the answer, not a cure, but insight into what the future could hold, and some assurance that my doctors will know what to do if my working diagnosis becomes real.
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