What is Juvenile Dermatomyositis

Juvenile dermatomyositis is an autoimmune disorder in which your own immune system attacks your muscles and skin. When breaking down the term dermatomyositis, derma in medical terms means skin, myo means muscle and itis means inflammation. The disorders first sign is often a skin rash that appears usually on fingers and eyelids it is rosey in color and sometimes turns purple. ( Emma's first presented on her face. She now has it on her hands, stomach, knees, elbows, toes, and eyelids.) In the beginning some people complain of feeling weaker.
It is unknown what causes JDM. Doctors think that in order to become sick with JDM a child needs to come in contact with one or more factors from their surroundings (called environmental exposure), including certain infections, sunlight, life stressors, or other stimulants of the immune system or muscle. ( Don't let this scare you, there is more needed. Your child probably wont get this from a day in the sun.) A specific combination of genes (probably many genes) is also needed. Genes are the inherited materials inside your body's cells (DNA) that make up your body and give directions for your body to work as it should. Because so many different things have to add up just right JDM is very rare. The frequency of rare diseases is difficult to measure. Doctors best guess, based on a recent study, is that at least 3 in 1 million children develop JDM each year in the United States. If this estimate is correct, then approximately 2 hundred to 5 hundred new cases will develop each year in the United States.

Tomorrow we will go over the symptoms in more detail.

This picture is of Emma at her worst. She was completely unable to hold her own head up! Still smiling!!!!!!

The Diagnoses

In order to do this blog right we have to step back in time to when all of this started. Emelia has not been well for a while. How long we are not sure. As we look back now, things that we assumed to be symptoms of "Not the baby anymore" might have been symptoms of something more.
When Emelia (known as Emma to her friends and family) was 18 months old we brought home baby number 5. Cutting her baby time down and making her a big sister. At this point we thought her want of reverting back to bottles and her desire to constantly be held was just for some missing attention she was used to getting. Now we wonder if we were wrong. As my husband always says "Hind sight is always 20/20."
In mid August we felt like something was not right.... but we had no idea what was going on.
Emma started not wanting to walk down the stairs in the morning or after naps. Not too odd.... maybe just some growing pains. About a week later her face broke out in a rash. A little concerning... nothing huge.... right?
The rash got worse so I took her to a prompt care place (her doctor couldn't get her in that day). They looked her over stating it was on her knees, stomach, hands and face. Diagnoses...eczema. 1 week later Emma had her well child check up. I spoke with the doctor about both issues. I felt that what I was seeing on Emma was not eczema. Emma was unaware of the rash. Never touching or itching it, not typical of eczema. I told the doctor this, also explaining I had other children with eczema and it never presented this way. Her diagnoses....eczema and growing pains. She did tell us that if Emma got worse with not wanting to walk they would x-ray her left leg. That day Emma received a vaccination in her right leg. ( I will tell you the importance of this later)
We are now in mid September, 1 week after her well child check up. She wont walk more than 20 yards and only wants a bottle... nothing to eat. I am really getting worried at this point!! We went ahead and had an x-ray done of her left leg....nothing there. Once again doctor says its growing pains treat it with Motrin and Tylenol. She says at this point if it continues she will refer us to an pediatric orthopedic surgeon. This was not enough for my husband and he tells her to refer us now. Our expected wait for this appointment was 2.5 weeks.
Last week of September Emma would not walk at all. She would not eat, only drink bottles. She couldn't roll over in bed and couldn't hold up her own head. She complained all the time of hurting... her elbows, her legs, knees, hands, stomach and neck. I took her back to her doctor. More x-rays of her leg and blood work....Nothing!!!! Her doctor tells us to hold out for the other doctor...he should be able to help her...
October 12, Emma went to see the pediatric orthopedic surgeon. He tells us "If I can't cut her open and fix the problem, its not what I do." Wow, that is what I call comforting...NOT!!!! HE tells us to see an actual pediatrician or a pediatric rheumatologist. The wait list to see a rheumatologist is ridiculous! Pediatrician it is!
Wednesday, October14 Emma went and saw her pediatrician. After looking her over for an hour... he has no idea... or at least not enough to pin it to one thing. His genuine concern for her was all I needed at that point. I felt that he would do as he said he would and call other doctors compile opinions and get back with us the next day.
Thursday, October 15 Emma was diagnosed by two doctors a pediatrician and a pediatric rheumatologist. Diagnoses....Juvenile Dermatomyositis. Next question.....what the heck is that????

This picture was taken on a bad day of Emma's rash. It is hard to ever see the rash in pictures but the pink she is laying on brings it out.