Let’s back up. Around St. Patrick’s Day Elia had a mysterious rash appear on her knuckles. At first we thought that she had irritated them by sliding her hands into her toy storage cubes and rubbed the skin. They just didn’t look like a ‘normal rash,’ but more like red blisters.
The rash was a tricky one because it would look like it was getting better one minute and then back to its original form the next. Then the irritation spread to the palms of her hands and her cute little feet. We had several appointments with her pediatrician, sent pictures back and forth, and had other doctors in the practice check it out and they were all stumped. Realizing that this may be out of their scope of expertise they referred us to a pediatric dermatologist.
On Friday, May 17th, we walked in to the dermatologist’s office expecting to be sent home with a stronger cream and be done with the whole thing. Unfortunately, that’s not what happened. I could see the concern on the doctor’s face as she asked questions and examined Elia. And as she said the words “biopsy” and “blood work” I knew that this was something much more serious.
There sat our sweet little Elia in her tiny little hospital gown, our healthy baby girl, a vibrant almost 23 month old who had never even had as much as an ear infection. I knew that the biopsy was going to be painful and confusing for her and I just couldn’t take it. I also knew that she wouldn’t understand why people that she didn’t know were holding her down–and why her Daddy was helping. I’m so thankful that he is so strong in situations like this.
I, like a big fat chicken, opted to wait outside with Zavier as I knew my fragile emotions (just 7 weeks post-partum) couldn’t handle seeing her in distress. I will say that I busted in the room just as soon as I could and gave her a big squeeze and told her that everything was going to be okay. We may have even promised ice cream.
Elia was such a trooper only crying for a couple of minutes after being let up from the table. She then picked a fun prize from the treasure box and was ready to get the heck out of Dodge. Unfortunately, we had to stop by the lab for the blood work and she had to endure more pokes.
We finally got the call with the results yesterday (on 5/22/13) and it confirmed our fears. Elia has been officially diagnosed with Juvenile Dermatomyositis (JDM). Don’t worry, we had never heard of it either. I still have a hard time pronouncing it and I’ve heard it over and over this last week. I’m confident that this is denial in its purest form.
JDM is very rare (about 3000 to 5000 cases in the US) and can be very serious. It’s an autoimmune disease that affects the skin and muscles. She’ll have it her entire life as there is currently no cure. The most mild cases are managed by medication and go into remission in comparison to more serious cases in which the patient can lose enough muscle strength to have difficulty (or lose the ability) to swallow, sit up, or walk. We’re not sure yet where Elia fits on the spectrum and our hope is that we have caught it early enough that we can manage it and hopefully she’ll go in to remission after her medication and therapy regimen.
She had an appointment with a pediatric rheumatologist today (5/23/13) and she explained her lab reports, our plan of action and and what to expect as we start this journey. The rheumatologist was very pleased with her range of motion and strength but she let us know that it’s very difficult to test muscle strength on patients as young as Elia. She told us that an MRI would be our best option.
Her treatment will include steroids, methotrexate, monitoring visits, and specialized therapies based on her needs. She’ll be admitted into Children’s Mercy on Monday for three days to undergo three steroid infusions via IV over the course of three days. She’ll also have her MRI to evaluate the state of her muscles and diagnostics to determine if her swallowing capability has been compromised.
Once she’s released from the hospital she’ll move to once a week steroid infusions and weekly methotrexate injections. Her steroids will be tapered off until they are removed completely. She’ll also undergo blood work and diagnostics throughout the treatment to monitor her progress. It’s possible that she may remain on the drug regimen for years with the average being 3 years. If needed, she’ll have a speech therapist, occupational therapist, and physical therapy. She’ll also be monitored by her pediatric rheumatologist and pediatric dermatologist. We have already been assigned to a team of doctors that we have been extremely pleased with.
In addition to her therapies she will be hypersensitive to sunlight so we need to exercise extreme precautions to make sure that she isn’t overexposed. She’ll wear sunscreen every day for the rest of her life even if she goes into remission as even minimal sun exposure like walking from the car into a store can cause a flare up. She’ll also need to wear a hat when she’s outside and those of you that know Elia know that this shouldn’t be a problem. Thank goodness our sweet diva loves a good hat. On sunny days she’ll need to be indoors during peak sun especially when she’s in the active stages of the disease and on her medication.
She’ll also need to be very careful with germs. We’ll need to do our very best to make sure that she’s not around someone who isn’t feeling well and learns to wash her hands well and often. Even the most simple cold or virus could wreck havoc on her system.
So, now that you know the what–I’ll tell you what I think about it. I think it sucks. I’m worried, confused,
angry pissed, but most of all sad for her. In a matter of days, less than a week to be exact, her life’s path has just been altered and there’s not a damn thing I can do about it. All we can do is try our best to make her comfortable, safe from potential dangers and do our part to make sure that she has the best healthcare that we can give her.
We realize that some of you are family and very close friends and we hate that you’re finding out this way. This has all happened so fast and we have been told that we need to move even faster in order to be aggressive and hopefully stop it in its tracks. We would have loved to have reached out to each and every one of you but it just isn’t possible.
I’m hoping to post with updates on her health as often as I can but am literally just trying to keep up with life in general with two under two and our new reality. If you’d like to learn more about JDM you can visit: the Cure JM Foundation. Just keep in mind that, as with any disease, there’s mild and severe and everything in between. As someone who has Googled nonstop for days–trust me, don’t. There’s just too much out there that may or may not apply to her case–the link above is a good general reference.
We’ve cried many tears, we haven’t slept in days, and just wish it would all go away.