Chicago Bound

It's official - we're heading to Chicago for an appointment with one of the foremost experts in JDM. I'm excited and relieved and nervous, but mostly relieved. I've done nothing but worry and second-guess the decisions around Mielle's treatment ever since we dropped our first rheumatologist (well, actually even before that - from the very beginning, truth be told); some peace of mind is desperately needed. I don't know if I will get it, but I feel like this is my best chance at it. I don't know if this will be a one-time evaluation or if we end up going regularly.

It will be a long day for the appointment, and I'm worried about how Mielle will take the whole experience. But I just found out that there is a nature center in Chicago with an amazing butterfly garden... Mielle is SUPER into butterflies and moths right now, so I hope that will help take the sting out for her.

We're flying to Chicago Aug 10, have the appointment on the 11th, and we will stay the 12th to have fun (butterfly garden!) so the trip won't be a total bust for Mielle. Our fantastic and amazing friend Stephanie has agreed to come along to help keep things light and help with logistics. Mielle adores her and I'm so, so grateful for her generosity. Blown away, in fact!

We're also hoping we may be able to connect with the lovely Kristin Connor, who currently resides in Milwaukee. Nancy Poozy is there, too, so maybe we'll see her, also. We hope!

Yard Sale-O-Rama

We're getting a lot of good stuff coming in for the sale next Saturday (July 26) - thanks so much to everyone! This whole project has done so much to lift our spirits and help something positive come out of this mess. The yard sale really has taken on a life of it's own, and keeps growing. We are getting drop-offs from friends as well as people we've never met. The folks at Mielle's former preschool, Home Sweet Home, had been planning a group rummage sale as a fundraiser for the school, and decided to direct all of that stuff to us. It's really been amazing, and it really, really touches us.

Here's a video Lenny did to advertise the sale:

http://www.youtube.com/watch?v=AVWSwbZe_1g

One of my visions for the yard sale was that it would provide a tangible way for the people that care about us to assist us, and reduce that terrible feeling of helplessness that we all experience in this situation. And I pictured it as a way to rally our community, bring everyone together and hopefully become a positive force for everyone involved. All of this is coming to pass, and HOW! It is way beyond my expectations!

It has also been a great way to get media coverage which will raise awareness about JDM. We are to be featured in the Alameda Journal on Friday, and we are working on other outlets as well. I also hope to have some coverage of the event itself - we'll be taking photos, of course! If nothing else, it will be covered HERE!

So, once again, Thank You to Dianne and Lisa for organizing, and Thank You to everyone who is donating stuff and helping out of Friday and Saturday. I can't really express what it all means to us.

Here's a family pic we took for the Journal:

trying to try something new

This one was sent mid-July 2008.

Hello, friends -

So, just a quick update - Mielle did the IV steroid treatments for a little more than two months, and has improved. Her skin looks better (less red), she doesn't complain of leg pain too much anymore, and her strength and energy have been improving. At the same time, she did seem to "plateau" with that treatment - it was helpful, but she reached a point where it didn't seem to be pushing her forward anymore.

We've discontinued the IV steroid for now, and started her on another oral immunosuppressant drug called cyclosporine. Some patients have had good results using this drug in combination with methotrexate, which she has been taking from the start. She hates the taste of the new medicine, but she realizes it's better than going to Stanford and getting an IV!

We are also considering another new treatment which she would actually take as part of a study. It's another drug that is delivered intravenously, and if we do it it would probably happen in the fall. We are currently trying to taper her oral prednisone (the drug that makes her gain weight and makes her head "feel crazy") because if she does the trial, all her other meds need to stay constant, and her doctor doesn't want her to stay on such a high dose of oral steroid for so long. So it will take some time to gradually reduce that med, while watching carefully to make sure that the disease doesn't break through again as a result. If it does, we may need to re-evaluate. But, we've been reducing for a couple weeks already, and so far, so good.

We're on the books with an appointment in August with a specialist in Chicago, but we are checking with that doctor now to see if it's a good time for us to go - since we are kind of in the middle of trying something new. It might be better to wait until we have a clearer idea of whether it's working.

In happy news, some friends are helping organize a big yard sale to raise funds for us, and for JDM research. It is really taking off - growing by leaps and bounds - and the response from the community has been incredible, to say the least! We are also looking to get media coverage to raise awareness and right now we're pretty certain that the local paper, the Alameda Journal, will feature the story; we're still working on other outlets. We are lucky to have a lot of savvy friends who know how to make this stuff happen.

And THANK YOU to everyone who made a donation to Cure JM in Mielle's honor! It was so great to hear about donations coming in. She has fallen a little behind in making her art pieces for donors, but they will come eventually! And I've just been informed that Mielle has set the Cure JM record for the most donations made in her name. Hooray to everyone!

If you meant to make a donation, but forgot, or want to give more, here's the website again:

www.curejm.com

I want to set up a blog which I can update more regularly, so loved ones can just go there to read the latest news rather than waiting for email updates, but just haven't gotten to it. Maybe I'll try to have it in place by the time we do the yard sale (July 26) so that I can share pics, etc. more easily.

much love,
Suzy

www.curejm.com

This was sent May 10, 2008. Our mounting frustration is apparent...

Hey there -

Another update, and a shameless fundraising appeal. (You've been warned!) And this is loooooong. Sorry.

Mielle did well with the iv steroid treatments. The first go-round was a series of three infusions on three consecutive days. Obviously, it would be hard under any circumstances, but the whole thing was made worse by the timing of our appointments - they got progressively later, and by the end we weren't getting out of there until nearly 10 pm. (The alternative to that lousy scheduling was to wait another week, which we weren't willing to do). However, Mielle handled it well, for the most part, especially after she was settled in with iv dripping, watching a movie.

Anyhow, we survived! And two weeks have passed, and she had another treatment a few days ago. Her skin is definitely looking better looking better, and we're hopeful this means the treatment is having a positive effect. She hasn't reported much change in her leg strength; she's functional but slow on stairs, awkward getting up off the floor, etc. The next step, if iv steroids don't do the job, is a treatment called IVIG, which is an intravenous infusion of immune globulins, a pooled blood product. Yes, it sounds a little creepy some have had good results with it. (And some haven't). The whole treatment plan is very hit or miss - "Let's try this combination of toxic chemicals, and if they don't work, we'll try a different combination of toxic chemicals, and if that doesn't work, we have this other combination of toxic chemicals we can try..." and on and on.

Sorry to sound so negative, but quite frankly, it stinks that there is not a better alternative to treat this disease (juvenile dermatomyositis -- nobody can pronounce it, much less remember it, so I'm giving the name here again. Here's how to say it - break it into parts: der-MA-to-MY-o-SI-tis).

Don't get me wrong - I'm glad to know what it is, and I'm glad that treatments exist, but even when they work as hoped (which is NOT all the time), these treatments are brutal. I've talked about the challenges of steroid-induced personality changes and rage (there are really no words to describe how it feels when your 5-year-old girl says that her brain feels "like it wants to explode out of my head and beat someone up"), and raging appetite and weight gain, but there are other complications that can happen - as a result of the treatment, not the disease - such as diabetes, high blood pressure, bone loss, and bone death. (I hadn't even heard of "bone DEATH" before. Nice.) Stunted growth (Mielle hasn't grown taller in months... but then again, the disease itself also causes stunted growth, so maybe I'm being unfair with that one!) Stretch marks. Thin skin. Cataracts.

I'm just trying to express that even when things are going well, it's awful to see, and live, and experience, the effects of these drugs. And when things aren't going well, it's indescribable. We are doing our best to stay positive and all that jazz, and I think we're doing ok at that. But I'll say it again: there needs to be a better option to treat this disease.

Which leads me to the shameless fundraising appeal. There is an outstanding organization called Cure JM, and it was founded about 5 years ago by two family members of kids with this disease, a mom and a grandmother (two different kids/families). They have done amazing, amazing things in this short amount of time. They have created a website with oodles of information, and a message board so people like me can communicate with one another. They organized the research and publication of a book that covers literally every aspect of this disease. They have awarded over $1.5 million in research grants, and funded a newly-opened clinic at George Washington University that is solely dedicated to the study and treatment of this diseases (as well as the adult version). According to the announcement press release, this clinic clinic was ENVISIONED DIRECTLY BY MOTIVATED PARENTS, AND MADE POSSIBLE BY A GRANT FROM CURE JM.

Since JDM (the "hip" lingo for juvenile dermatomyositis) is so rare, it doesn't get much funding, etc. and so it's up to us to make it happen. Cure JM has shown what is possible, and they are working hard, and effectively.

Just so you know, there is currently no cure at all. People often aske me if Mielle will outgrow this, and the answer is "no". Here's the breakdown: About 1/3 of afflicted children will eventually achieve remission after some number of years of treatment (2 at a minumum, 6 or 7 at maximum), and will never have a recurrence. About 1/3 will achieve remission, but will suffer one or more relapses, and when that happens you start all over again at the beginning with the treatment regime. These relapses (called "flares" in the lingo) can be triggered by something as simple as a sunburn (a SUNBURN!! I mean, come on!!), are often more severe than the initial onset, and they can occur years after remission. About 1/3 will never get to remission, and will always need to be on meds to try and control the disease. All of this is just to say that a child does not outgrow the condition, and basically the uncertainty about whether it may recur will always be there. This needs to change; we need a cure.

Back to Cure JM, and money - it is completely run by volunteers, mostly parents of afflicted kids, so literally ALL of your donation goes to funding research, providing support to kids and families like ours, and raising awareness about the disease.

So, you can see that any money donated to this organization will work hard to help Mielle, and other kids like her, and many other kids who are worse off than her.

You can check out the website:

www.curejm.com.

Don't be put off by the (in my opinion) cheesy intro! You can read lots more about what they're doing. It's all good. There are lots of prominent "click here to donate" links, so I'm confident you can find your way! (And of course, it's tax-deductible).

And, extra-special bonus -- everyone who makes a donation, of any size, in Mielle's name will receive a personal, handcrafted thank-you gift from the hands of Mielle herself! She makes super cool art and you will be a lucky owner of an original piece... maybe a drawing, maybe a finger-knit, maybe a little sewn doo-dad, maybe a popsicle stick creation. Who knows?

If you don't want to donate to them directly, you can still do something to help. There is an organization called igive.com, and if you register with them and click through them first when you shop at tons of online stores (all the biggies!) then a percentage of the $$ you spend goes to Cure JM. Here's the link:

http://www.igive.com/welcome/warmwelcome.cfm?c=30238&m=0

Honestly, this one is a no-brainer. It's so easy, and it can help assuage your guilt about spending too much money at Old Navy!

Friends, I apologize for the long-winded post. I've been composing this appeal in my head for months, and it feels good to finally put it down and send it out. Thank you for all your love and support (and I apologize that there are many email responses from y'all, from the last update, that I have not responded to personally -- yet! I appreciate them all - but we're just doing the best we can over here...)

We are scheming about a couple of fundraising type events for the local yokels (a huge yard sale, maybe some kind of musical event) - not sure but will keep you posted. And in the meantime -

PLEASE DONATE!!!

PLEASE DONATE!!

Finally, what shameless fundraising appeal would be complete without some sappy images to tug at your heartstrings? Here they are. The first is a shot of a poem Mielle wrote for school. Allow me to translate:

When I grow up I will be a artist
I will work the hardest as a artist
I will paint the ocean blue
To share them with you

xxooo

Suzy

Setback

This one was sent around mid-April 2008.

Hi,there -

I apologize that I haven't sent out a report on Mielle's condition for a while. I was having a hard time sitting down to do it, even when things were progressing well, because it's all so very difficult and complicated and emotional... and then, just after I finally wrote an update and was about to send, things changed.

After about 4-5 months of steady progress, Mielle is currently suffering a relapse. It's not as bad as it was in the beginning, but the muscle weakness and pain is definitely back.

Although we previously had a lot of confidence in her rheumatologist at Children's Hospital, we'd begun having doubts and now that she is having this setback, we felt he wasn't being responsive enough to her condition. We decided to seek a second opinion at Stanford. We took Mielle there yesterday, and have decided to move her under their care. We agree with their assessment that her relapse indicates a need for more aggressive treatment. She's still on the oral steroids, and a few weeks ago we switched to giving her the methotrexate (which also suppresses the immune system) weekly by injection. (Previously, she got that orally, as well).

In addition to this, she'll be having intravenous infusions of steroid, hopefully next week (IF she can get on the schedule). Initially, it will be a series of three infusions on consecutive days. Then one infusion every two weeks for 2-3 months. Then, spacing out to every 4 weeks, and so on.

We'd hoped we wouldn't have to go to this more invasive treatment, but the disease is obviously not being controlled with her current regime. I read that one dermatomyositis expert said something to the effect that if the oral steroids are a handgun in the fight against this disease, then the iv steroids are a bazooka. We need a bazooka.

"The experts" also indicate that early, aggressive treatment is key in achieving remission. The idea is to slam the, uh, heck out of the disease at the outset. If treatment is not aggressive enough, it can actually sort of "train" the immune system to continuously flare up again whenever the meds are tapered, and create a situation where the disease becomes more chronic. We don't want chronic, we want remission.

There are other things that can be tried, if this doesn't work as well as it needs to. But we are hoping this will be as extreme as we need to get.

Attached are recent pics. You can obviously see the physical changes that the steroid has created in MIelle, but I feel like you can also really feel the turmoil and struggle that is going on in her body, when you look at her photographs. It's heartbreaking. But her spirits are good. We still have some issues with the steroid-induced rage, etc. but it hasn't been nearly as bad as in the beginning. The second shot is with Uni, Mielle's rock-star friend who was kind enough to come over and spend time with her a while back.

Lucien is just dreamy, a little angel whom we all adore. He's doing a lot to help all of us through this hard time. We are grateful.

Please continue to keep us in your thoughts and prayers.

So far, so good

Update # 2, around the end of November 2007, after about one month of treatment -

Hello friends,

Just wanted to update everyone on Mielle's situation. I won't lie, it's been a challenging month dealing with the meds she is on. The prednisone really messes with Mielle's temperament, making her mood swing wildly (and I mean WILDLY). Super hyper and manically happy one moment, completely falling apart in pieces the next. It's been exhausting trying to navigate through this each day, but we're managing and lately we have had some better days. Not sure if that's just a "some good days, some bad days" kind of thing, or if things are actually improving in a meaningful way. The doc says the "moodiness" (understatement!!) goes away after "a few weeks", but then again he never even told (warned) us about that particular side effect in the first place, and he talks about it in that "pooh pooh" kind of way that old-school doctors have, as if it's no big deal in the first place, so... I'm skeptical! But I still hope that he's right!

The other med she's on, methotrexate, is only once a week and seems to really knock her out when she takes it. We figured out that it's better to give that one late in the day so she can just konk out and go to bed.

Okay, so that's the bad news. The really, really good news is that we are getting the feeling that she is responding to the meds. Just in the last few days, she has been noticeably stronger in very small, but very tangible ways. It is a huge relief to see some results, however subtle. We know that it is going to take time for her to get her full strength back, but it's really wonderful to see some positive signs that she is on the mend.

One weird development: when speaking with the school nurse about this, she told me that there is another girl at Mielle's school that has this disease... !!!! I've spoken with the mom on the phone, and we have plans to get together and probably get the families together also. Their daughter just went off her meds three months ago, after three years of treatment. She was diagnosed right before she turned 4, so she's 7 now and in 2nd grade at Mielle's school. Her little brother is in kindergarten there, but he's not in Mielle's class. Anyway, this mom (Michelle is her name) seems very ready to reach out to us, and I think it will help us tremendously to share experiences and see a real live example of another family that got through this situation, and is now on the other side of it. Mielle is excited to meet Kendall and I hope it will help her as well.

We are so blessed in our community of friends. You have rallied around us in a way that really squeezes my heart. Thank you so much to everyone, for your love and support. We are feeling every bit of it, whether you are here in Alameda and stopping by for play dates, or you're far away and sending your love and positivity to us from a distance. We feel and appreciate all of it.

It's hard, but we're managing. It still feels very surreal at times, that this is happening to her and to us. It's really bizarre to be pumping such powerful pharmaceuticals into her tiny little body, but at the same time I'm so thankful that we have a treatment for her. It's a love/hate relationship with the treatment, I guess.

I really hope that the darkest days are already behind us; now that we can actually see some signs that the meds are working, things definitely feel more hopeful. If Mielle responds well to the treatment, then we should be able to begin reducing the prednisone dose at some point relatively soon, hopefully not longer than two more months from now (although we'll be increasing the methotrexate, which has it's own set of side effects... but supposedly not nearly as intense as the prednisone). We just have to hope that we can move through all of this as quickly and smoothly as possible. Right now, we're taking things one day at a time, and each day brings us closer to the other side.

diagnosis

Mielle was diagnosed on Halloween of 2007, and here is the email I sent out a couple of weeks later to let people know. It's kind of hard to read it now - the original rheumatologist we saw painted quite a rosy picture, and we were more than willing to believe his version of the story. Unfortunately, things have not been as simple as he lead us to believe. Here's the email:

Hello, dear friends -

We have some news about Mielle's health that we want to share with her close friends.

She is okay!! But she's had a weird rash on her face for months, and although we took her to a few different docs, none could explain what it was. Then about three weeks ago she began complaining of pain and weakness in her legs. I had been pretty mellow about the rash, but when the leg pains began I got really freaked out and feared it could be lupus (which exists in my family). Once we recognized that she was having the pain and weakness in her legs, it seemed to progress quickly. Within days, she couldn't climb up stairs without pulling herself up with the rail; she can't get up or down on the floor unless there is a piece of furniture she can use to lean against; she can't do a sit-up; she can't walk more than a couple blocks without getting fatigued.

To make a long story short, we were referred to a pediatric rheumatologist at Children's Hospital in Oakland and he was able to take one look at her and diagnose her with a disease called juvenile dermatomyositis.

Basically, it's inflammation of the skin and muscles, exact cause unknown, but believed to be related to genetic predisposition and some kind of trigger that sets it off - environmental, sunlight, food, immunizations... who knows? It is not contagious in any way. She has a textbook case.

Here is a link to a good info page on the disease:

http://www.muscular-dystrophy.org/information_resources/factsheets/medical_conditions_factsheets/juvenile.html

So, the good (GREAT) news is that we know what it is, and although it's serious, it's also manageable. More good news was that she could begin treatment at home, and didn't have to go into the hospital for 2-4 days of i.v. medication.

The bad news is that she has to take prednisone (a super powerful steroid) for at least three months, perhaps longer. She should start to feel some improvement in a couple of weeks, and be back to full or nearly-full strength in 2-3 months. After that, she will need to continue treatment for quite some time (2-3 YEARS) but hopefully with less intense meds as time goes on. If all goes well, she will eventually be medication-free and in remission, but it is one of those things that never completely goes away so there is the chance it could recur. However, the rheumatologist was very optimistic that it could be almost-cured.

This disease is very, very rare (only 3 kids out of a million get it every year, can you believe that??) and the more I think about it, the more I think we are unbelievably lucky to have found a doctor who could immediately recognize it, and has a lot of experience treating it (over 100 kids). Thank God for Children's Hospital! It's the type of thing that can easily be misdiagnosed (often for lupus) and treated incorrectly, or simply not recognized by the treating doctor, resulting in months of testing, etc HELL. So we are incredibly grateful to have dodged all of that, and to have her started on the necessary treatment.

We will also be taking Mielle to see Sally Savitz, our wonderful homeopath/acupuncturist - a miracle worker if there ever was one. I know that Sally's treatment will help Mielle deal with the disease as well as the medications she needs to take. I feel very blessed to have Sally working with us.

Mielle is in pretty good spirits most of the time, although she is frustrated that she can't run and play at recess and she does get tired and grumpy very easily (but hey, nothing new about that!!) So far the prednisone has been having some noticeable effects on her mood, causing her to be (at times) irritable, hyper, and prone to mood swings. Hopefully that will decrease as her body adjusts to the medication. We have taken her out of the after-school-care program she was in, so that she can come straight home and eat, rest, etc in the afternoon. The rheumatologist suggested pulling her out of school for a couple of weeks, but we really didn't want to, and neither did she, and so far she seems to be doing fine with it. Obviously, we've alerted her teacher, etc. so they all know what she can and can't do right now.

So... I think that's it. It's been pretty rough and very scary, but it's getting better. It's actually a LOT better this week than it was last week, when we didn't know what it was and when we first got the news. It sucks that this has happened to her, but there are so many ways that it could have been so much worse, so we're doing out best to look at things from that angle. We appreciate all the good vibes you can send our way.

Getting the Blog up...

Okay, here we go... hoping this will make it easier to keep everyone updated.