Weak muscles and skin rash are the main symptoms of JDM, (What Taylor has) while muscle weakness without a rash is the main symptom of JPM.
JM is a rare disease, what causes this disease is unknown. Approximately 2 to 4 children in a million in the United States are diagnosed with JM each year. That is 2-4 TOO MANY!!!
JM begins in childhood or the teen years. For some reason the average age a child is at the time of diagnoses of JDM is between six to seven years old; 25% are age 4 or less. JPM usually develops several years later.
JM affects girls twice as much as boys. Once a child is diagnosed with JM, it is always considered to be the juvenile form, even if the patient continues with the disease into adulthood
The immune system is a group of cells that normally protects the body from infection and other environmental factors. In an autoimmune disease like JM, however, the cells get active and they cannot stop. The immune system ends up attacking previously healthy tissues, harming the body instead of protecting it. In JM, the immune system mistakenly targets muscles, skin and other tissues.
The immune system attacks healthy tissue in JM this is called inflammation. In JDM patience, the inflammation focuses in the blood vessels that lie under the skin and in the muscles. With JPM, the attack is focused more against the muscle cells.
This inflammation causes the weak muscles and – in the case of JDM – skin rashes. If blood vessels or muscle cells in other parts of the body are inflamed, other systems of the body can be affected, such as the digestive tract, heart and lungs.
Although medications can help ease the symptoms of JM, the disease has NO CURE.
The primary medications used to treat the symptoms of JM are corticosteroids, immunosuppressants and chemotherapy. These medications themselves can cause severe side effects, making JM challenging to treat. Many researchers believe that early and aggressive treatment is usually the best predictor of a better outcome of this disease.
High dose oral Prednisone or other corticosteroids often coupled with intravenous corticosteroids (Solumedrol) are usually the first line of treatment for JM. Since the side effects of corticosteroids can be very troublesome, Methotrexate (a chemotherapy drug administered at much lower doses than used for cancer patients) is usually introduced early to allow for tapering of the corticosteroids.
Second-line treatments include Cyclosporine and Intravenous Immunoglobulin (IVIG). Plaquenil is often used to control the rash.
For patients who do not respond to the first and second line of treatments, or in patients severely affected by the disease, Rituximab, Cytoxan and CellCept can be added. Protection from UVB is essential with sunscreens. Vitamin D with adequate calcium intake keeps bones strong.
These medications all have their own side effects, but the most common ones for Prednisone are:
- Increased appetite and weight gain
- Rounded face
- Mood changes
- High blood pressure
- Stretch marks
- Fragile bones and bone damage
- Cataracts
- Slow growth
In addition to medications, physical therapy is generally prescribed to increase strength and flexibility in muscles and joints.
There is no cure for JM, but with advances in early diagnosis and aggressive treatment, the outcome has continued to improve. Some children experience a mild form of the disease and may go into remission. Others follow a more severe and potentially debilitating course that can be life-long. Some JM patients will have a loss of range of motion. Some will battle an array of serious complications, resulting in the inability to walk, ongoing pain, disfigurement and even death. Whether the course of the disease is mild or severe, JM is life-changing for all of these children and their families.
INFO gathered from Curejm.org
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