Happy Thanksiving! (a bit tardy..I know)

Please excuse the delay. I was forcefully and violently sucked into the vortex of holiday upheaval and have finally been spit out on the other end. After taking the first few days of this week to dust myself off, I have decided to resume writing and get to the thanksgiving post I have been thinking about since November. While I agree that it is rather odd to be sending out thanksgiving wishes on January 5th, for those of you who know me well.....6 weeks late is really not so bad. I have been feeling intensely that there is something not right about missing thanksgiving on my blog and I have known that when I have the chance to return to writing, thanksgiving would be the first order of business. You see, over the past months, I have felt many feelings surrounding Sophie's journey with JDM. As one might expect, I have experienced feelings of fear, sadness, loss, loneliness, guilt, overwhelm and pity. However, the one feeling that I have been fortunate enough to be experiencing as of late is that of thankfulness.

I have so very much to be thankful for. I have access to experienced doctors who were able to diagnose Sophie quickly and, in turn, treat her effectively. I have good health coverage and, as a result, have been able to afford critical evaluations, treatments and medications in a timely manner. I have a father and a father-in-law (both doctors) who have been there to help with medical questions. I have a tremendous support  group among my friends, family and community who are there for me. I have a husband who is truly a partner in parenting our children...in sickness and in health. I have a handsome, healthy, smart, sensitive and caring son who has gracefully risen to the challenges of having a sibling who has required a bit of extra attention. And, I have a beautiful, strong, funny, courageous, bright daughter  who is responding well to her treatment and growing stronger everyday.

There is something about coping with illness in a family that suddenly puts everything into perspective. In an odd way, that is something to be thankful for.

So what if the post is 6 weeks late?!  We are here, we are healing and we are hoping. We are hoping that 2011 will be a year of health and happiness FOR ALL.

So with thanksgiving behind me, I can NOW wish you a VERY HAPPY NEW YEAR!!!!

Bear Country

Just returned from Bear Country last night (a.k.a. Cubbie County, Blackhawk Belt etc). You Chicago sports-fans know what I am talking about. Chi-town, the Windy City....call it what you like, I am becoming more and more of a fan of that beautiful city by the lake. For one, when I am there it feels as if I am no longer  behind enemy lines. You see, raising a Chicago sports-fan in NYC is no easy task. Throughout his childhood, poor Zachary has been steered away from Yankees caps, shuttled past Rangers t-shirts, rushed by Jets pencil cases and veered away from countless other items of NY Sports paraphernalia. Our family is relegated to online shopping for our sportswear needs, whether it be at chicagosport.com or wrigleyvillesports.com. Luckily, I am rather adept at online shopping, but it sure is nice to walk into a store in Chicago and find our favorite teams represented on the racks. Somehow, you do not feel as alone. As in, 'Hey! There are OTHER people who like the Cubs?! Guess what Zach? We are not alone!'
But aside from the comraderie that Chicago provides, this week I am happy for the good news that it has provided. Sophie and I flew to town on Sunday afternoon so that she could visit her Dr on Monday morning. The Dr has reported that she is very pleased with Sophie's positive response to treatment thus far and seemed quite optimistic about her prognosis. She took a blood sample and explained that if Sophie's numbers continue to look good (especially after her recent reduction in prednisone), she would feel comfortable taking Sophie down to 17 mg of oral pred and then slowly decreasing the dose 1 mg at a time. Hallelujah! Two airline flights within 24-hours in the company of  a 4 and 1/2 year old on steroids is not an easy feat, but I would travel just about anywhere to get that kind of good news. The fact that we were in Chicago, surrounded by "our people" (grandma and grandpa included), makes it that much sweeter.

Top Ten List

Top Ten Signs Your Child is Suffering from the Side Effects of Oral Prednisone

10. She goes to bed at night planning breakfast

  9. At breakfast, she plans lunch

  8. At lunch, she plans dinner

  7. She breaks into impromptu songs about H & H bagels and how she likes to eat them with cream cheese

  6. She can often be found sitting in her window gazing longingly at the Chirping Chicken

  5. When you wipe her face to get the chocolate ice cream off she exclaims, "Hey, I was gonna eat that!"

  4. You are on a first-name basis with the pizza delivery guy

  3. Its not unusual to awake at 3:00 am to find every light on in the house and see her wandering about 

  2. Upon departure of any trip you happen to take, even just down the street to the market, "Are we there yet?" is heard and then repeated with escalating volume and intensity in two minute intervals until the mission is either completed or aborted out of complete frustration

And the #1 sign that your child is Suffering from the Side Effects of Oral Prednisone?

...You are awoken from deep slumber in the middle of the night by the doorbell  to find your four-year-old in the company of the night watchman who informs you that he found her wandering the lobby in her nightgown!

As you can plainly see, the side effects of Sophie's medication have been intensely challenging. As a result, we are thrilled with this week's blood test results. Her muscle enzymes continue to look good and the Dr has given us the go-ahead to reduce her from 22.5 mg of prednisone to 18.5 mg. We are very relieved to be moving her medication in this direction. The Dr also mentioned that Sophie's glucose was a bit elevated. He thought it might be due to the prednisone or stress, but I am quite certain that it is more likely due to the econo-size box of candy dots she inhaled before the blood was drawn. Chalk it up to another prednisone success story!

Little Things

So...(insert deep breath here)...our NYC doctor finally returned from vacation and we met with him to discuss "the plan". Apparently, our expectations were too low. We were simply hoping for his willingness to work with the Chicago Dr and "get aggressive" if need be, but we did even better than that! He said, "Sophie's blood work looks great and her numbers are improving across the board. 'Chicago' AND I are BOTH comfortable continuing with the current treatment (with a couple minor tweaks)." Phew! Now that IS good news! We feel like we dodged a bullet there. Hopefully, her numbers will continue to improve and the Drs' treatment  differences will continue to be a moot point.  

As for Sophie, she is starting to ask a lot more questions about why she has to give blood, get shots and take so much medicine. I know a frank conversation with her about her disease is imminent. I just feel I have to be ready to put on a brave, can-do face before we have that talk. After all, what help can I be to her if I, her "fearless leader", am gasping for breath from a paper bag?! So....(insert additional deep breath here)... this is my journey. I am working on coming to peace with our "new reality" so that, in turn, she can.  I have to say, today it's looking pretty good.  Sophie is happy at school doing everything a four-year-old should be doing--painting, block-building and snacking on a steady diet of goldfish and graham crackers. She is happy at home with her family playing Candy Land marathons and singing aloud to her favorite songs.  And, she is particularly happy with her new shiny patent leather shoes. Sophie is doing fine.  In fact, it is becoming quite clear that we would all be better off around here if we followed HER lead. Like all children, she knows something that we adults all-too-often forget. It is the little things.

Bravery

Nooooow I get it. I am a little slow. I could not understand why I was being commended for bravery in writing about Sophie's illness, but now I understand. When you are a blogger, its not always about the I-know-exactly-how-I-am-going-to-pummel-this-disease-to-the-ground, grind-my-heel-in-its face and stick-my-flag-in-it-times. It's also about sharing the frustrated, exhausted, doubtful and scared-out-of-your-mind times. After all, this is the point of the blog. This is what makes it real. If I hold true to my intentions of sharing (See my entry Sophie Myn, points 2,3,4 and 5), then I have to share it all. What is the good of things, kitchen sinks and fingernails aside, that are perfectly edited, manicured and sanitized? Life is messy. And at times, as we have discovered this month, very very scary. 

The last few days have been the hardest we have had since Sophie has gotten sick. Ironically,  it has nothing directly to do with Sophie. Rather, it has to do with the fact that it seems our doctors in New York do not agree with the treatment advice we have gotten from Chicago. (Our regular rheumatologist is away on vacation until next week so we will see what he says when he returns, but his partner has made it clear that he absolutely does not agree with the aggressive treatment advice we received and will not move forward.) Apparently this is a very common problem in Juvenile Dermatomyositis (JDM), as there is no standard protocol for treatment. As far as I can tell, this is due to a couple of reasons: 

1) There is not a lot of research on JDM, as there is a small population to study  (3 out of a million children get JDM). Therefore, it does not attract a lot of attention or funding.

2) There is tremendous variability in JDM from patient to patient.

As a result,  JDM is not very well understood. That's right, folks. I have had a hard time getting my head around that myself. So let me repeat it. THE SAME DISEASE THAT FIVE WEEKS AGO HAD MY DAUGHTER TOO WEAK TO HOLD HER OWN HEAD UP IS NOT WELL UNDERSTOOD. There is little agreement between doctors, their assessment tools vary (Chicago used different tests than New York to assess disease activity in my daughter) and treatment protocols vary. So where has this left us? Aside from a state of deep despair? It has left us in a position of having to make complicated medical decisions that the world's best doctors can not even agree upon. Do we work with the expert in Chicago who has dedicated her life to researching and treating JDM patients or our talented pediatric rheumatologist who belongs to the practice in my neighborhood that has cared for my children since birth? My hope is that these doctors can somehow find a way to work together that will make all of us feel comfortable. And if they can not do that? Then I guess RON AND I will just have to do whatever it is WE think is best for our little girl. Talk about bravery.

The Starting Line

July 2010 was heaven. I was enjoying a beautiful summer with the kids in the Berkshires. Zachary learned to roller blade, Sophie learned to swim. There were hikes, bikes and countless hours treading water in the pool. It all came crashing down in August when Sophie began to complain about being tired. As the month wore on she became increasingly fatigued and was not even able to walk fifteen steps without needing to take a break. By the end of the month she was not interested in participating in any activities, could no longer dress herself, run or climb stairs. By the time September rolled around, she began to drop things, fall while walking and became so weak that she actually asked me to hold her head up for her while I washed her hair. On September 2, 2010 she was diagnosed with Juvenile Dermatomyositis (JDM) a rare auto-immune disease that affects her skin and muscles. Turns out that the rash that appeared on her leg in January 2010 was not excema after all, but rather an early sign of her disease--one that is easily missed as it looks similar to excema. Sophie was put on Oral Prednisone (steroids) right away and began to receive weekly injections of Methotrexate (a chemotherapy drug) in an effort to shut down the inflammation in her body, quiet the immune system and turn the disease around. In addition to all of the medication she was prescribed, in one week's time she also had the following tests/procedures: an MRI, several blood draws, a muscle biopsy, a swallow study, chest x-ray, pulmonology tests and a four-day stay in the hospital for IV steroid treatment--that is A LOT for a little girl (and her parents)!!! After one week on the oral prednisone, Sophie began to feel better and has been rebuilding her strength and stamina daily. She started school this week and is thrilled to be back with her friends and teachers. However, I started this blog today because I have gotten the clear impression that fighting JDM is a journey. We have a good chance of sending this disease into remission with aggressive treatment, but there is a lot of variation between patients and there is no cure. If we are lucky, we will send it into remission in the next two years and never hear from JDM again. That is our plan. To that end, after getting word from Sophie's Dr in Chicago (where we took her last week to be seen) we will be ramping up our treatment plan. Based on the labwork that was done last week, the Dr feels that the disease is 1) further along than we had originally thought and 2) still active in her muscles. As a result, she has recommended that we surgically implant a port so that we can infuse Sophie with IV steroids two times weekly. Fine with me. As far as I am concerned this is simply another way to get the medicine into her body. The worst is behind us. We already have the diagnosis. Now we have a plan. Wish us luck!

Sophie Myn

On September 2nd, 2010 our four-year-old daughter, Sophie, was diagnosed with juvenile dermatomyositis (JDM), an auto-immune disease that attacks her muscles and skin. I have decided to write about it. Here is why:
1. It is a practical way to update concerned friends and family on Sophie's health and treatment.
2. It is therapeutic.
3. It is not secret or shameful to have a disease.
4. Although it is rare, we are not the only family to have a child diagnosed with JDM and there is a chance that we can provide information, solace or "connection" for others by sharing our story.
5. I have found that when you share your thoughts, feelings, fears and dreams, AMAZING things unfold--and you discover that you are not alone.