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!
On Thursday, July 22, 2 days before we left for Chicago, Trip had a swallow study done at Arkansas Children's Hospital to determine if there was any dysphagia involved with his JDM. Our speech pathologist was wonderful with Trip and he had a blast blowing bubbles, drinking "chocolate milk" and eating a Bareo- an Oreo with barium laced filling. They observed 1 mis-timing of laryngeal closure, 1 incident of midlevel penetration, and multiple normal swallows. There was no aspiration or pharyngeal residue with any swallows. According to the therapist and radiologist this was very good and they didn't make any recommendations or suggest any change of diet at this time. They said the 2 findings could be random incidents that are even found in perfectly healthy individuals. Then, on Saturday while we were on the road to Chicago I got a call from Dr. Pachman just touching base with us before his procedures on Monday. I asked her if she had had a chance to review the results of his swallow study (they were immediately faxed to her from ACH) and she hadn't. I told her that the findings were very good and explained what was observed. She immediately said, "He should have zero (midlevel penetrations or mis-timings)! In a JDM patient we have to assume that these findings mean something. All the better that we're moving forward aggressively." She means business and doesn't mess around with this disease. I like that!
