A Lot to Swallow

This Thursday, July 22, Trip will have a barium swallow test done at ACH. Lately, he's been coughing and clearing his throat some when drinking liquids, which can be an early sign of dysphagia (swallowing problems). He's been drinking less and sometimes just refuses to drink anything. Dysphagia is a somewhat common problem in JDM patients when the muscles involved with swallowing start to weaken. Given that we already know that he has weakness in his neck, it makes sense that there might be weakness in the muscles inside the neck and throat. It's also necessary that he do a barium test so that we can know if there are any problems that could affect anesthesia next week. He has recently had a little bit of a head cold so there is a possibility (and we do hope) that this is all related to drainage and actually not dysphagia. But, again, the good news is, if he is suffering from dysphagia it should quickly be resolved once he begins steroid treatment.

And speaking of beginning treatment, here's the current plan (I'm 95% sure that this is how it will go. I should get a call tomorrow from Pachman's office confirming this): We're planning to drive to Chicago this Saturday, 7/24, so we can have Sunday to have some family time and enjoy the city- visit the Shedd Aquarium, Lincoln Park Zoo, etc. And, yes, Ruby is going with us. Monday may be a long day for her but the daily infusions aren't going to take as long as we originally thought. On Monday, Trip will be put completely under with full anesthesia (not just conscious sedation like the last MRI) and have an MRI which will immediately be read. Then they can do the muscle biopsy and port placement while he's anesthetized.

After surgery on Monday he will likely go on and get his first SoluMedrol (an intravenous corticosteroid) infusion. There is a downside to getting his first infusion right away. I'm not sure why this is, but the way the port works, if you don't immediately access it within the first 24 hrs. after surgery (which Pachman doesn't recommend doing) you have to wait a week to access it. So, even though he'll then have a port, he'll have to get those first 3 infusions the old school way. I'm hoping that they can use the IV placement from the surgery and maybe even just tape it down when we leave the hospital each day, so he doesn't have to get repoked each day. If not, he can just get the EMLA (numbing) patch pre-poke even though poor little man is going to be awfully tired of needles coming at him. He'll get the 2nd dose on Tuesday, 3rd dose on Wednesday, and we'll drive home afterwards on Wednesday or Thursday.

The infusions should take about an hour, so that's why we're going to go on and take Ruby. I've heard of some JDM patients taking IVmeds that took 6 plus hours so I feared that that was what he was getting at first. That would've been way too long of a day for Ruby day after day. We pray that the side effects from the meds will be minimal and Trip will feel up to doing a few fun things in Chicago on Tuesday and Wednesday. We'll just have to play that by ear. The most common quick side effects of the SoluMedrol are a severe metallic taste, headaches, and anxiety and mood swings. I've heard some people call it "PMS in a bag!" Yikes!

I think the current health prayers for Trip are pretty self-explanantory: that he will have a successful surgery on Monday with little discomfort to him, and that the infusions will be affective with little to no side effects.....and safe travels for us as we make the 20+ hour round trip car drive yet again! And thank you God that the air conditioner on our van went out last week, so that we could get it fixed, and not next week during the Chicago trip. That would've been horrible!!

Ice Cream Truck!!

There's nothing much more nostalgic in the summer than getting a treat from the ice cream truck. This past Monday morning Ms. Ice Cream Lady made a visit to the park while we were there with MMM. They heard the tune of "Do Your Ears Hang Low?" the moment the truck turned into the park and then came running. Maybe I just need a little recording of that song to play so they'll come when I call them. Hmmm....

Co-Sleeping

When Rex and I check on the kids before heading to bed ourselves, we often find that Ruby has been quietly busy in her room after she's been put to bed. What we found this night was particularly cute- the baby doll cradle right next to her in the bed. What a sweet little mommy!...... btw, Ruby's head is on the unicorn Pillow Pet that she got for her birthday. Thank you, Aunt Becky!

Ruby's (and Trip's) Special Day

Since we spent Ruby's birthday at home recovering from the quick Chicago road trip, the next day, July 9th, was Ruby's Special Day. She wanted to go to the cupcake store and then go see "Despicable Me." Ruby, you're wish is my command!
Cupcakes on Kavanaugh even had extra special tiara cupcakes.

The movie was really cute. Happy Birthday, sweet girl!

The Chicago Update

Sleeping babes, just before arriving in Chicago.

Well, I think we're finally on the road to recovery from our long Chicago journey, but Trip's road to recovery is a much different story. We saw Dr. Ibarra, Dr. Pachman's fellow, 1st. Ibarra walked in the room and from several feet away looked at Trip and said, "Oh yeah, he definitely has dermatomyositis" (from noticing his facial rash, eyes, and hands). On one hand, it was wonderful to finally have someone that was very familiar with what was going on and that didn't look at him like he had 2 heads. But on another hand, it was very frustrating to discover that it could've been diagnosed so quickly and easily, though our docs here took 6 months of poking and prodding him to figure it out. Last summer when I showed his facial rash to one of the pediatricians in our clinic she said to change laundry detergent. Uhhhh, he doesn't wear clothes on his nose!

Dr. Ibarra then did a very full examination of Trip and very quickly discovered that he had weakness in his neck. She did various muscle strength tests including asking him to lie flat on his back and then lift his head off the table (just his head without using shoulders or abdominal muscles). He couldn't do it. He was clearly trying to , but just couldn't. That discovery alone was worth the trip to Chicago. As his mother, I strongly suspected that there was more going on than just a rash but didn't know how to pinpoint it. She then gave us instructions on how we needed to be more careful with Trip since he didn't have full strength in his neck and would be higher risk for a spinal injury: no trampolines, roller coasters, slides at Wild River Country, or any heavy rough housing. We also have to keep him in a backed carseat until his neck strength returns for added support if we were in a crash. I'm a carseat Nazi so this was no biggie for us anyway. I couldn't help but wonder, why hadn't anyone done this basic muscle strength test? (I know- I sound like the commentator from "Mystery Diagnosis.") I know that this is a rare disease but it's not as if we had to run some bizarre series of blood tests to figure it out. She saw a facial rash and then asked him to lift his head. Ugh!.....Ok, I've said my peace and I'm over it now. We're moving forward! Loss of neck strength is often one of the first things to go with JDM and one of the first to return so Trip is very lucky that we've caught it when we did. His prognosis is very, very good and with treatment any muscle loss should return to normal and his rashes will disappear.

They then did a very full examination of his nailfold capillaries which is one of the main ways they track the disease. The JDM Research Clinic there at Children's Memorial Hospital has the only device in the country that can photograph close up images of the nailfold capillaries, so that they can have a record of how they look from one appointment to the next. The nailfold capillaries tell the story of what is likely going on in the capillaries throughout his body. We'll be going to Chicago atleast every 6 months so they can photograph his cuticles. How funny!

When they looked at Trip's they saw "drop out" which means there are disconnects in the capillaries. This told them that he definintely needs intravenous meds, not oral meds alone. He likely has these drop-outs inside his intestines so he wouldn't have optimum absorption of meds taken orally. So, how does a 3 year old get IV meds? He will have a port-a-catheter surgically implanted in his chest to make this easier. Whenever he gets meds or needs a blood draw, they'll just take it from the port rather than having to poke him numerous times. We're hoping that his weekly infusions can be done via home health instead of going to the hospital weekly and sitting there for hours. We'll be working out the details of this soon.

Dr. Pachman (or Dr. Wiggle Waggle, as she calls herself) came in, examined Trip and confirmed all of Dr. Ibarra's findings and then let us just bombard her with questions. She's a very likeable lady and just repeatedly thanked us for bringing Trip to her when we did. They also explained to us that the MRI performed on Trip at ACH in Little Rock was incomplete. They looked at the records from ACH and the MRI was ordered correctly but somehow didn't get completed exactly as ordered- something to do with "full fat suppression." They weren't seeing a full, clear image of his muscles and there could possibly be inflammation that just wasn't visible from the current MRI (another reason I'm glad we went to Chicago). They also want to do a muscle biopsy but without a good MRI image, they won't know where would be the best place to take the muscle sample. He'll have to have another MRI, but this is an insurance snafu that we're trying to work out. The muscle biopsy should give them all of the answers we're needing to know about Trip that haven't been answered with blood test alone. The muscle biopsy results will tell them what meds he needs to be on. I know that he'll atleast be on Solumedrol (a cotricosteroid) and probably a host of other things. She does want us to continue the Plaquenil which Trip has adjusted to remarkably. It is horribly bitter, despite being custom-compounded into a grape simple syrup. I touched a tad of it to the tip of my tongue and had to immediately grab something to drink. What works for Trip is for us to quickly squirt it all into his mouth and then give him a piece of sugar free bubble gum once it's down. He does it with no problem so he can get his gum!

Trip was also seen by physical therapy who did more specific test for weakness. He did fairly well scoring a 39 out of 52 on the CMAS (Childhood Myostis Assessment Scale) with the primary problem being in his neck. She gave us some exercises to do to help strengthen his neck muscles and to help him shift the weight on his left foot which is slightly turned inward.

Since this blog is now also serving as a journal for Trip's disease process I want to mention muscle enzymes. Our LR docs keep checking for an elevation in Trip's muscle enzymes (he's always in the normal range). Dr. Ibarra explained that, for some unknown reason, JDM children that have gone untreated for long periods of time, will have muscle enzymes that return to the normal range even though they actually do have muscle involvement. Unless you check muscle enzymes early in the disease, muscle enzyme blood test won't accurately reflect muscle involvement.

Trip has an appt. July 29th for a consult with the surgeon in Chicago and then to have the muscle biopsy and port placement surgery the following day. Pachman strongly encouraged us to have this done in Chicago since the surgeon that they use is very familiar with JDM patients and the technique that she prefers for the muscle biopsy. We're also hoping to get the new MRI scheduled for the 27th or 28th in Chicago once insurance approves it. Once his port is in place he'll receive his first infusions there in Chicago to see how he handles them. Pachman usually does 3 days in a row of infusion and then weekly thereafter. We won't know the details of his therapy until after the biopsy but we do know to plan to be in Chicago for about 5-7 days this next trip. Ruby will likely stay with my parents in Memphis. Ruby and Trip have never been apart for more than maybe 12 or 15 hours. They will be lost without the other. =(

Our current prayers are this:

1. That insurance will quickly approve an additional MRI

2. That Trip will not have any significant change in his condition before we begin treatment (I'm a little paranoid right now that every little thing is the disease worsening)

3. That the surgery and meds will be a success with little side effects.

4. Praise that we followed our instincts in seeking further care for Trip. I'm so glad we made the haul to Chicago!

Happy Birthday, America!

On the 4th, we waited until late afternoon when the sun had calmed down and then went to Wild River Country. Ruby wore her patriotic swimsuit. She appears to be channeling Elektra from American Gladiators!
Trip has been such a good sport with the sunscreen applications and hats this summer. Such a sweet boy!

Rocking the Edward Cullen look!

After WRC we rushed home to shower, get the kids in PJs and head downtown for fireworks. Trip had to take his new Buzz flashlight and Ruby her butterfly glow stick.

We again went to our great fireworks viewing spot in a parking garage downtown. We just opened up the hatch of the van and watched from the comfort of our car.

Ruby took this picture of Rex and me since I remembered we had a similar photo from several years back.

This was taken atop the then TCBY tower on July 4th, 2003 just a couple of months before our wedding. We were just little chicks back then! Happy July 4th to all and to all a good night!

Farewell, Avery

On June 19th Ruby went to a little going away party at The Painted Pig for her best preschool bud Avery. Avery's family has moved to Jonesboro so her Dad can begin practicing at a hospital there.
The bright pink cupcake icing was a big hit since it doubled as lipstick.

Best wishes in your new home Avery! Ruby will really miss you!

Pool "Day"

I'm playing blog catch-up today. A couple of weeks ago we met the Days at the pool that they frequent in North Little Rock.

We all sported matching suits. How cute are they?

Happy 5th Birthday, Beautiful Girl

I can't believe that it was 5 years ago today when I suddenly leapt out of bed around 8:30 a.m. when my water suddenly broke. Rex says, "Are you sure? Really?"....."uh, yes. I'm sure. Urine didn't suddenly just gush from me in my sleep." It was a Friday and Rex hadn't gone to work yet because I had been having some inconsistent contractions and suspected that it might be the big day. She was due July 5th and my doc was planning to induce the next week if I didn't have her over the weekend. Ruby Sheaffer Reeves graced our world that night around 8:30 p.m. and our world has never been the same..........Above, Ruby is enjoying her birthday morning surprise, a My Little Pony nursery set. She had to arrange them just right for the photo.

I was in love with her from the first second and love how this picture captures my joy!

And look how grown up she's looking now. I took this July 4th at WRC.

Ruby, you are stunningly gorgeous, inside and out and we love you infinity (as you would say). You are so smart, witty and talented and have one of those infectious giggles that one cannot hear without smiling and laughing right along with you. I thank God every day for our precious gem, Ruby. Happy Birthday, sweetheart!

A "Toy Story 3" Birthday

On June 25th we had a birthday party for Ruby a couple of weeks early so that we could see Toy Story 3 at The Rave Theatre soon after it's release.
Whoa! That hat makes Ruby look extra tall.

We were joined by the Days (Vivian and Bauer) and several Maumelle neighborhood friends: Abigail & Tyler Simpson, Malia & Grant Bugg, Danny & Drew Mayer, Gavin Brzezinski, & Lilly Crandall.

The Maumelle Kroger Bakery did a great job on the cake!

The 5th candle is hiding behind Jessie- yes 5 candles! I can't believe it! Thank you friends for helping us celebrate! Ruby had a wonderful time!

An Update on Trip

Last Thursday evening, after the official diagnosis had been made, I joined the parent's discussion board on CureJM.com and spent hours just reading about other parent's stories and questions and answers between parents of kids with dermatomyositis (from this point forward I will be abbreviating this to JDM. Schwew! That word is a doozie!). What I consistently saw were parents recommending to other parents that they should take their child to see Dr. Lauren Pachman in Chicago. Some even said things like, "We wish we had seen Dr. Pachman sooner. Her treatment plan quickly changed the course of the disease and our child may not have had to suffer so long had we gone sooner." I shared this with Rex and we both agreed that we should go on and try to get an appt. for Trip in Chicago- not only for Trip's sake but for us as his parents. We could have the peace of mind knowing that he was seeing the expert and would be getting the best possible care.

Because the disease is so rare, there aren't many doctors who have a lot of experience with it. I think our pediatric rheumatologist here at Arkansas Children's Hospital is doing the best he knows how to do with the limited experience that he's had with JDM. I haven't asked how many JDM patients he has yet, but I'm guessing that it couldn't be more than a handful or 2. Dr. Pachman is one of the world's foremost researchers and top experts on this disease. Her practice is dedicated to JDM children and she has seen hundreds of these patients.

So, the next morning (Friday, June 25th) I called Dr. Pachman's office in Chicago to make an appt. for Trip, explaining that he was a newly diagnosed JDM patient. They penciled him in for July 19th while they started the process of getting copies of all of his records from ACH. The following Tuesday morning (June 29th) I got a call from Pachman's nurse just saying that they had received Trip's records (kudos to ACH for that speed!) and needed to ask me a few questions before they confirmed the appt. In my conversation with the nurse I discovered a couple of things that made me a little uneasy about our current plan of treatment with Dr. Dare. First, we chatted about Trip's MRI. I said that it was normal accept for some findings in his buttocks that my dr. didn't think were of any significance. He said that something in the legs would be consistent with JDM. She explained that that was actually what they were looking for on the MRI- a symmetrical abnormality in either the leg or buttocks. Hmm? (I've since then gotten the CD of the MRI and the finding in his butt is on both sides) Then I asked her if I should continue to give Trip the Plaquenil or hold off until we saw Pachman in a few weeks. She said, "just Plaquenil? I've never heard of a patient only being on Plaquenil." Ok. I began to slightly panic. Clearly, our current plan of action wasn't aggressive enough but I didn't want to contact Dare with my concern. I wanted Pachman to be the one to decide how to treat Trip. I decided that all we could do was pray that his condition didn't worsen before the 19th.

Then, 5 minutes later I got another call from Pachman's office in Chicago wanting to know if we could be there next week, July 7th (they're only in clinic on Mons & Weds, completely booked on the 30th, closed for the holiday on the 5th). Dr. Ibarra, the fellow, had just reviewed Trip's case, and thinks he should be seen ASAP. She said that this disease has been brewing in him for quite some time and that he should've been diagnosed months ago. Also, that she wasn't comfortable at all with him only being on Plaquenil. Thanks God! Someone that recognizes a real problem and wants to proactively do something about it!

Meanwhile, I've been reading the book "Myositis and You: A Guide to Juvenile Dermatomyositis for Patients, Parents, and Healthcare Providers" partially written by Dr. Pachman. A sentence in the book jumped out at me last night: "Skin disease in JDM is associated with inflammation of blood vessels and should be treated as aggressively as muscle weakness with both systematic medication and topical agents." From reading this book I've gathered that he'll probably be put on an intravenous corticosteroid and methotrexate as this seems to be the preferred treatment at diagnosis. They may want to put in a port-a-catheter to make administering these drugs easier since he is so young. I'll update after we see Pachman on Wednesday..... as of now, not sure how we're getting there. Round-trip plane ticks to Chicago are now $500+/person. We may be making a quick 2 day haul to and from Chicago via Honda Odyssey. Ugh!