Two weeks ago today we took Elia to the pediatric dermatologist and everything changed. That is, everything except our sweet, beautiful, brave Elia. Since being diagnosed with Juvenile Dermatomyositis (JDM) she has been poked, cut, examined, starved, medicated and put through things I’m not even sure I could take and she has handled all of it with grace and strength. She continues to amaze us on how brave and ‘big’ she can be.
I’ll pick up where we left off with our last post.
Our stay at Children’s Mercy.
Monday (5/27/13) we checked in at Children’s Mercy for a three-day stay. I’ll say that our drive to the hospital was so incredibly tough. I’m not even sure Zameer or I said a word. We sat there in silence as she sat in the second row of our minivan watching a movie. She was singing and dancing and was as happy as she has always been. I can’t say for sure what Zameer was thinking but I know that I was thinking that here we were taking our (what appeared to be) happy healthy daughter to the hospital and she had no idea what was about to happen. We knew that the next few days would be uncomfortable for her and that she would have massive amounts of medicine pumped through her tiny little 24 pound body. We could only hope and pray that we were doing the right thing and that she’d forgive us.
When we arrived at the hospital she had no fears because she’d not only never been to the hospital as a patient but had never even been sick. The hospital was decorated with bright colors and animals on the wall and she probably imagined that we would be having a fun day. Unfortunately for all of us that was not the case.
She had her IV placed on the first day and was such a brave girl. She only cried during the initial poke and then watched closely to make sure that they were doing it correctly. Once her IV was placed they did her first steroid infusion and she did so well. It didn’t seem to bother her at all as she just sat on my lap and watched a movie on her iPad. The infusion only lasted about 45 minutes. After that was done she was free to play with all of the toys that they brought her but was confined to her room throughout her stay to minimize contact with germs.
The first night was tough as the steroids give her a boost of energy and she was exhausted but couldn’t physically slow her body down long enough to fall asleep. Our little girl was dialed up a few notches and the only thing that would calm her down was us holding her and pacing in our little room attached to her IV pole. So, her daddy and I took turns holding her and walked the room over and over all night long. She finally fell asleep about 6 am only to be woken up around 7:30 for vitals.
To add to her frustration she couldn’t eat or drink after 1 am for her procedures that weren’t scheduled until 2 pm. Trying to explain to a 2-year-old that she can’t eat or drink (sorry baby, no gilk) is nearly impossible. She was so sad and would take her tiny hands to our face and say “gilk” with her big brown eyes and we had to say no. Heartbreaking times a million. She was taken down to surgery at about 1:30 pm and we were glad that we were moving along and maybe getting in a bit early. As nervous as we were for her to be sedated we knew that it had to be done and she would be that much closer to getting to be with us and eat and drink. She was scheduled to have an MRI and have her port placed and both required sedation.
At the very last minute and due to a crazy situation with an allergy of mine (would she have it too?) we almost had to delay the procedure another day or two. After consulting with an allergist and immunologist we all felt comfortable moving along as planned and were only delayed an hour. She did great with both procedures and was wheeled back up to her room about 2 and half hours later and greeted us both with hugs and kisses. We were so happy that we were able to give her some milk and it wasn’t long until she was up running around and working on her puzzles.
That night she had her second steroid infusion and her first dose of methotrexate via her port. The methotrexate process scared me a bit more than I had imagined. After watching the nurses take extreme precaution administering the drug I noticed that it seemed so much more intense compared to her other medications. I know that it’s a low dose cancer drug and have even had to have a dose myself after delivering Elia and struggling with retained placenta. BUT, I had to ask…why was this so different? The nurse told us that if they were exposed or if it touched their skin it could have harmful side effects. That’s when I broke down. They were afraid of a drop of it touching their skin and here we were pumping it in to our 23 month old daughter with a plan of doing it long-term. The nurse tried her best to assure me that her body needs it to get on the road to remission. All I could think of again was…”I hope she can forgive us.”
Because of the day that she had and the fact that her steroids may have kept her up all night again we opted to give her something to help her sleep as she needed the rest more than anything else.
Day three (our last day) had another frustrating start as she couldn’t eat or drink in order to prepare for another test. We first thought the test would be at 8 am which would have been wonderful, then told 9 am which wasn’t awful…unfortunately it didn’t happen until 1:30. Another day of no food and no gilk. Poor baby.
After her test she did her last steroid infusion for the week, had a little lunch, and took a nap. She was finally allowed to leave the room for more than a procedure and rode a tricycle around the floor. I’m pretty sure we made 20 laps and neither her daddy or I may ever walk upright again.
We then worked with her coordinator to set up home health for her weekly infusions, an occupational therapist on some possible therapies, and were finally dismissed with a stack of papers and a huge bag of medicines and supplies. Our heads were swimming our eyes were glazed and we were exhausted…but we were heading home. And we knew that’s exactly where we needed to be.
The results are in.
Her MRI showed NO, ZERO, ZIP muscle damage (cue music and happy dance). This means that the disease has not affected her muscles (yet). It means that the rash was the first sign of the disease. We are so, so, so relieved. With this disease the rash can appear before, at the same time, or after the muscles have suffered damage. Some children go years with the muscles becoming weaker and weaker before the rash appears. It looks like we caught it early enough that we may be able to avoid any serious damage. Elia will be monitored closely going forward so it’s not to say the disease won’t affect her muscles but with monitoring visits we should know before significant damage and weakness takes place. Huge sigh of relief.
We decided to go with a port for her infusions to avoid weekly IVs. Even though she doesn’t appear to have any muscle damage her rash was pretty significant and it will still take an aggressive treatment plan to get her into remission. Hopefully the worst is over in regards to the port and it will prove to be the easier method for her.
The labs that we have received back have all improved since her initial labs with the dermatologist. There was only one enzyme (liver enzyme) that was elevated but it was minimal and had decreased significantly since her first lab work.
Overall, her team of doctors are very pleased with her results, as are we. We may be looking at a diagnoses of Amyopathic Dermatomyositis (AMD). AMD is an even rarer form of JMD in which the child has the skin rash but not the muscle weakness. AMD is SO rare that there isn’t much research or information. It’s tough to diagnose, especially this early, because the muscle weakness may just not have appeared yet. So, as of now, they’re looking at AMD but if the muscle weakness appears her diagnosis would change back to JMD.
We were told that AMD is tough too and that we aren’t ‘out of the woods’ yet. But, we’re taking the fact that we caught it early and that she doesn’t have any muscle damage as huge wins and trying to be positive. And, right now…that’s a BIG deal.
She’s home and seems fairly comfortable. She takes several medications daily (Vit D, Calcium, Folic Acid, Zantac, Prednisone, and Protopic-a topical cream for her rash). She’ll have her steroid and methotrexate infusions once a week for awhile until they start to down dose. We’re going to Children’s Mercy on Monday for her infusions and then going forward we’re hoping she’ll start doing them at home with a pediatric nurse. The duration of her treatment is still unknown.
We also found out that she can play outside (and even in summer clothes) as long as she is properly sun screened, wearing a hat, and she avoids peak sun times. She has to wait three weeks because of her port, but can go swimming in the late afternoons/evenings.
We are SO pleased with her results and SO SO thankful for her team of doctors. We consider ourselves very lucky.
A big thank you to all of you that have posted comments, emailed, texted, called and shared Elia’s story. We’re so grateful for all of you and know that this journey would be so much harder without all of you.