New year, more progress

Mielle and I went to Chicago again for an appt on Dec 9. The trip was a raging success in many ways - we had SO MUCH fun with Amy, who once again flew from NJ to spend time with us in Chi-town, as well as Kristin and Nancy who made the treks from Madison and Milwaukee, respectively, to see us. But more importantly, the doctor appointment went really, really well.

Everyone was thrilled with how well Mielle looks, and I got a little emotional, thinking of where we were a year ago, 18 months ago, two years ago... ugh. For a while there, it was looking uncertain as to whether we would ever get to the other side of this thing. And of course, we're not on the other side - yet. But things are looking better and better for our chances of getting there.

I vividly remember our first appointment in Chicago, when Mielle was a giant steroid puffball, her skin was a mess, and she was completely miserable in every way. She looked terrible, and she felt terrible, both physically and emotionally. Appointment day is a long day, especially the first-ever, and it was all I could do to help her get through the day with minimal outbursts of rage and/or crying and/or screaming. We were prepared - we had tons of activities (including a special backpack loaded with fun stuff that our friends from Mielle's pre-school had given us) and snacks, etc. but it was still incredibly difficult. Mielle did NOT want to be there, and she made it clear. She barely spoke (unless grunting and growling counts) and she'd play with one thing for a short while, then fling it away and thrash around on the examining couch, ripping up the overlying paper to shreds. The room was a complete shambles by the time Dr. Pachman actually made her appearance, and Mielle looked like a wild animal. It was horrible. I was barely holding it together myself, between trying to manage Mielle and my own emotions, while still absorbing all the information presented, asking the right questions, etc. I remember this sensation, this feeling of "how did we ever, ever get HERE?"

JDM is the only disease treated at that clinic, so when you see another family there, you know they have the same thing. I remember another mom and daughter there that day. The girl looked a little older than Mielle, and there were no outward signs of the disease, or side effects from meds. She wasn't unhappy; she was calm and pleasant, having a conversation with her mother. They simply seemed normal. While for us, things felt about as far from normal as you can get.

I could feel the other mom looking, trying to catch my eye and start a conversation, share experiences and perhaps offer support, but I just couldn't return the gesture. I felt on the verge of breaking down at any moment, and had to focus all my energies on simply holding it together. Somehow, that openness from another mom, that act of kindness, was too much to bear. I just... couldn't.

All of this was running though my mind during this last appointment. This time, Mielle looked great, felt great and (while she is still on a truckload of intense medication) things mostly seem normal again. It was all smiles from the various people passing through - the nurses, the physical and occupational therapists, the volunteer parent liason Jacqui, etc. Mielle was a little impatient about being there, but she answered questions, cooperated with the exams, and was mostly concerned with running to the window to look at the falling snow (exciting for a California girl!) and speculate whether it would cause Amy's flight to be delayed. When one of the nurses mentioned almost in passing, as she left the room, that Mielle looked great and "we need to get her off some more of these meds", I cried.

The door stayed open when she left, and across the hall, in another exam room, I could see a boy around Mielle's age, with his mom. He was very cushingoid, puffy and cross, flaming red cheeks, not talking, and the mom was laying on the exam table with him, just trying to comfort him. It broke my heart.

As we were leaving, we passed yet another family in the hall. Another sad-eyed child, exploding from steroids. Another prent, this time the dad, looking haunted and grim. We got on the elevator together, and I looked hopefully at the dad, trying to catch his eye, give him an encouraging smile, maybe share experiences. But he couldn't look at me. And I utterly, completely understood why.

So, the fight goes on. Mielle is doing wonderfully, for which (obviously) I'm extremely grateful. But our journey is not over, and really never will be; things could change, and quickly. We hope we are on our way to remission, but even if we get there, this thing could come back, because we have no CURE.

And in the meantime, there are other families... heartbreaking in their familiarity. And since many kids actually struggle much more than we have, suffering many more complications and setbacks... they are also heartbreaking in their differences.

So I won't stop running, and fundraising, and doing whatever I can to support Cure JM; their work truly reduces the suffering of families like mine. And unfortunately, there is no shortage of families that need the help.

Taper continues...

Mielle's latest labs looked good, and we decided to concentrate on getting rid of the prednisone first, so we are resuming our taper...

Fingers and toes crossed. If all goes well, she should be totally off the prednisone by Christmas time. That would be a lovely present, indeed. And it all keeps going well after that, we'll be able to stop the weekly injections of methotrexate and switch to an oral dose. But that feels like I'm getting a bit too far ahead. One day at a time.

Another Chicago trip, already

Lenny and Mielle went to Chicago again last week, and while we are still waiting for the results of the bloodwork, things went really well. Her doctor was pleased so we will resume the slow, slow tapering of her meds once we see that there are no surprises on the labs.

Mielle looks great and acts great these days. She has strength and energy to keep up with her friends, and her appearance has gone back to almost-normal (she is still round in the face and has a little poochy tummy, which I attribute to the steroid that she's still on. It's a MUCH smaller dose, but still, it's something). Actually, our next decision is which drug to concentrate on tapering next: the prednisone, or the methotrexate.

Pros to tapering the prednisone: reduce the steroid side effects, such as the puffy face and belly, mood swings (they are SO much better but we still see the effects now and then), resume growth. (Steroids stunt the growth; Mielle did not grow one bit taller for nearly 1.5 years. In the last few months, the dose has been low enough that she has edged up a little. It's wonderful to see her growing again, and if we reduce the prednisone even more, we presume this would speed up.)

Pros to tapering the methotrexate: we would soon be able to switch from a weekly injection (not fun, even after doing it for so long) to oral. She currently takes a number of additional meds, whose sole purpose is to thwart the side effects (debilitating headache and nausea) so as the mtx dose lowers, we would be able to reduce those as well, and have her on less medications overall.

Obviously, the goal is to eventually taper/eliminate BOTH prednisone and mtx, but we need to determine which takes priority right now. Dr. Pachman asked Mielle what bothers her most about having this disease and not being "normal", and she said she doesn't like being so short. So although we haven't totally decided, I think we're leaning toward trying to completely eliminate the prednisone.

Again, this is all dependent on her labwork looking good, so... fingers crossed.

And may I add that I am beyond ecstatic that these are the types of questions we need to answer now... "Which med to taper next, since things are going so well?" as opposed to "Which horrible drug should we TRY next, because things aren't going so well?" Believe me, it feels wonderful to be on this side of the fence, and I just hope, hope with every fiber of my being, that we stay here. Forever.

Mostly Good

Phew! Doctor visit over, blood draw OVER!

I'm exhausted but happy and relieved that there were no earth-shattering revelations at Mielle's appointment today. Her specialist here was pleased that her strength has improved in most areas, her skin looks good, and her spirits are better. (During our first visit to see this doctor in August, Mielle would do little more than grunt or growl in response to a question from a medical professional. She literally thrashed around on the table, ripping the paper on top to shreds, making messes with all the games etc. we brought to entertain her. She was basically a wild animal, and the room was a disaster area by the time we finished - thank you, prednisone! So in the area of "good spirits", there was nowhere to go but up...)

One little dark spot is that the doctor thought she could see small cataracts in Mielle's eyes. This is a side effect of the prednisone. She was checked back in September and was all clear. We need to have her seen by an opthamologist again to confirm or deny our specialist's suspicions. She did say that the cataracts "should" reverse once she's off the prednisone, but it was a pretty depressing moment in an otherwise encouraging visit.

Actually, something really wonderful happened today. Mielle still has a terrible time with blood draws. It usually ends with her screaming her head off while her arm is held down and it is ridiculously traumatic for everyone. We have tried a lot of things, and none of them have helped - until this visit.

A "Child Life Specialist" is someone who comes and explains procedures in a way kids can understand, maybe models the procedure on a doll, suggests coping mechanisms, etc. etc. We've often had one but, honestly, they've never been able to get through to my girl and really make a difference. She'd go through everything with them and gladly accept the stuffed animal and other little trinkets offered, but when the time came for the actual draw, we were back to square one; and a that point, (once the screaming begins) ain't nobody gonna make things better. So I think I can be forgiven for my skepticism when we were told that a Child Life Specialist was on hand.

Her name was Kathleen and she sat down to talk to Mielle about the draw. She asked about what had happened with blood draws in the past, and what might make it better. She and Mielle actually made a list of things to do that might help:

















Then we headed to the "draw" room. There was a room set up just down the hall, with a phlebotomist waiting for us. (This meant that we didn't have to go to the regular lab down in the basement, register, wait, and have all that extra time to get even more nervous.) I was still skeptical that this time would be any better, but despite some initial resistance, Mielle handled it much better than I could have imagined - and it was definitely a smaller number of vials required than the last time I took her to Chicago (that was our very first visit and it was something like 30 vials). What a relief... I didn't realize how much anxiety I had about the blood draw until it was over!

Here are some other highlights of the day:


















Mielle took this one of Gundy.
























A snuggle while waiting...



















Getting assessed by the physical and occupational therapists. Mielle's strength has improved, all except her core and abdominals. (We need to work on those).



















Her unicorn, a gift from Uni and her Ukelele (Mielle's special rockstar friend) offered some comfort. Thanks, Uni!!!

























Taking microscopic photos of the capillaries around her cuticles. I think that Chicago Children's is the only place that has one (and CURE JM helped fund it!!). It provides another clue as to the actual state of the disease in the body. (Bloodwork alone is often NOT a good indicator of disease activity, so we have to look at an array of things to try and asses what's happening.)




So, all in all, we have to be happy with where things are. It's still a long road ahead, but things are so much better than they were a year ago, six months ago, three months ago. We're grateful.

breathe and run

I was exchanging emails this evening with Suzanne Edison, whose daughter Flora also has JM. We met at the event in Carlsbad - I vividly remember seeing Suzanne and Flora cheering on Mamajoggers at, I don't know, mile 8-ish of the race. A part of the course where there weren't many spectators, and no other Cure JM folks. They were like a breath of fresh air at that moment when the 5 remaining miles of the race seemed waaaay too long.

Anyhow, I mentioned to her that despite the fact that everything seems to be going well for Mielle, the upcoming Chicago visit has me feeling pretty anxious. Her reply took my breath away, because it so precisely captured the muddle of feelings that I'm experiencing right at this very moment:

"Of course. It is so damn hard. The not knowing, the anticipation, the hope, the not wanting to go into despair, not wanting to be TOO hopeful either because falling off the "Hope Wagon" is awful. It is hard to find any place to rest. I think the Buddhists want us to find that middle ground where we hold suffering and joy as equal. But I am not very evolved.

breathe and run, I guess that's the best we can do for now."

Wow. Wow. She really, really nailed it. It certainly is hard to find any place to rest in all this. I am afraid of being too hopeful, and simultaneously afraid of falling into despair. Clearly, I am also not very evolved...

But I continue to breathe and run, breathe and run, breathe and run....

Chi-Town, again

Please check out this blog post from a JDM dad:

http://blogonkevin.blogspot.com/2009/04/milestones.html

Really, really amazing. Made me cry (a lot) and does a great job of expressing some of the emotions inherent to dealing with this disease.

I'm preparing to take Mielle to another appointment in Chicago this week. I really can't believe it's been over 4 months already since the last trip. The rapid passage of time is beginning to freak me out! Mielle is continuing to do well, as far as I can tell (the pinkness I was noticing a while back didn't seem to amount to anything, thank goodness), and her Stanford doc is really pleased with her progress. So I'm hopeful that the news will be good this week, but... it's nerve-wracking nonetheless.

After successfully reducing her methotrexate dose so that it is more in line with her current weight (she has lost a lot of her steroid weight but the dose of the mtx had never been adjusted for that), we resumed tapering her prednisone. She's now on 3 mg/day (as opposed to 30 mg/day at the beginning). I am so grateful and relieved to have her on a lower dose of this drug; she still suffers some side effects, but it is nothing compared to how unbearable things were, for her and for us, at the higher dose. My understanding is that if all continues to go well, we will keep on tapering that drug until she is OFF it. I suppose I will find out more about that possibility at the appointment this week. Fingers and toes crossed. It would be quite a milestone to have her finally off the steroid, after more than 18 months.

Lucien is such a little walker now; he seems to consider it his job to walk laps around and around the house (with a big happy grin) from morning til night, picking things up and putting them down in random places as he goes. Although he is still significantly delayed in all areas of his development, he is progressing in other areas, too - he has a lot more words now, and mimics us all the time. (He recently learned "hungry" and he really likes that word a lot!) We recently added a new therapy to his repetoire - Occupational Therapy, which focuses on fine motor skills (stuff he does with his hands). It keeps us hopping to maintain his schedule of services, but we know that all the intervention he's receiving is critical to the progress that we've seen, and we're so grateful that it is available to him.

Mr. Delightful continues to explore his emerging stinky-two-year-old side, but he does it in such an adorable and endearing way that truly, it just makes him that much more delightful. Except when he looks deeply into my eyes, grabs a handful of my hair, and tugs HARD while simultaneously telling me "NO!". That is actually really, really annoying. Still, I'm already thinking about how much I'll miss him when we are in Chicago.

On the bright side, as far as our trip goes, I'm going to have the chance to see some old, dear friends - Nancy Poozy, Kristin, and Amy. Amy is flying to Chicago to meet us on Wednesday, after M's appointment, and we'll have until Saturday to play! Nancy and Kristin are driving down from Milwaukee on Thursday to spend the day with us. I am very, very excited to see them!

Wish us luck at our appointment.

Family visit

Having fun with the fam. Nice to see all the kids hanging out! The big ones are so sweet with Lucien. He's just trucking all over the place, making messes everywhere he goes. Mielle's having fun, although having lots of people around all the time makes her a little... uh, edgy. And the kids were both up til past 11 tonight due to jet lag. Ugh.

Mielle has been looking a little pink to me lately, so I'm keeping a sharp eye on it but hoping it's nothing. (Pink could possibly equal inflammation, which is bad, bad, bad.) But overall, all is well. Pics soon!