Thursday, January 28, 2010

Good article...Good news!

Mortality rates for pediatric rheumatology patients significantly lower
Largest US study to date finds mortality outcomes improving

A recent study by researchers from the Cleveland Clinic found that the overall mortality rate in the U.S. for all pediatric patients with rheumatic diseases was not worse than the age and sex-adjusted population.

Furthermore, mortality rates were significantly lower than reported in previous studies of rheumatic diseases and conditions that are associated with increased mortality. Details of the study appear in the February issue of Arthritis & Rheumatism, a journal published by Wiley-Blackwell on behalf of the American College of Rheumatology.

The Childhood Arthritis and Rheumatology Research Alliance (CARRA) estimates that 300,000 children in the U.S. suffer from some form of arthritis or rheumatic disease. According to CARRA, childhood arthritis is the #1 cause of acquired disability in children and is the 6th most common chronic childhood disease.

While rheumatic diseases present well-known risks to health, function, and quality of life, several conditions—juvenile rheumatoid arthritis, childhood systemic lupus erythematosus, dermatomyositis, various vasculitides, and systemic sclerosis are associated in various studies with a small but significant increase in mortality.

The Cleveland Clinic study team, however, maintains that previous mortality studies were relatively small, reported mortality outcomes only on specific diseases, had a follow up time of less than 10 years, and were mostly conducted prior to the 1990s, when new and improved drug treatments emerged. The team suggests that larger studies may also be flawed because most were based on physician surveys, without strategies to verify response.

To determine the mortality rates, risks, and causes of death associated with pediatric rheumatic diseases in the U.S., the researchers examined the world's largest rheumatology registry, the Pediatric Rheumatology Disease Registry (PRDR), which includes 49,023 patients from 62 centers who were newly diagnosed between 1992 and 2001. Identifiers were matched with the Social Security Death Index censored for March 2005. Death certificates, referring physicians, and medical records confirmed deaths. Causes of death were derived by chart review or from the death certificate.

After excluding patients with malignancy, 110 deaths were identified among 48,885 patients in the PRDR registry. This number was significantly lower than the expected mortality from the age- and sex-adjusted U.S. population especially among 18,111 patients who were followed up for at least 9 years. The standardized mortality ratio was notably greater for systemic lupus erythematosus and dermatomyositis but not for systemic juvenile rheumatoid arthritis and was markedly less for pain syndromes. Most of the deceased with inflammatory disease died of their disease or disease complications, while many of the deceased with pain syndromes died of non-natural causes.

"One possible cause of the increased survival in the present study compared with previous studies may be the improved treatment that was introduced in the 1990s, said the lead author of the study Philip Hashkes, M.D., M.Sc. "Since the information in the PRDR was limited, we could not explore in depth for risk factors or early predictors of mortality. This and continued follow-up of this cohort for mortality trends should be investigated in future studies."

source: eurekalert

Monday, January 25, 2010

I believe!!!

I feel like I am dreaming....Saints to the SuperBowl!!! I do believe in the "Who Dat Nation"!!! Somebody pinch me!!!!

If you want to see the "spirit" of our state and how much it means to all...check out this video. I confess, we were all holding hands together in our living room and begging, pleading for the kick to be good. We all starting jumping, dancing, high-five'in...I cried!!!

Friday, January 22, 2010

So Proud!!!

Madeline won 2nd place for her lyrical ballet solo in Baton Rouge this weekend. How proud we are of her. As for her team dances - they won 2nd place in jazz and tap. We are so proud. And doesn't she look beautiful.
Now if I can only figure out how to post the actual dance on this site!!!
Slumber party tonight - I have 8 little 10 and 11 year olds coming's going to be loud and fun!!!! Pics soon!!!

Friday, January 15, 2010

Competition Time!!!

We leave for Baton Rouge today for Madeline's dance team's competition. This is her first year to compete with a exciting. She is doing lyrical ballet for her solo and then tap and jazz for team. Wish us luck!!!

Tuesday, January 12, 2010

Happy 11th birthday baby girl!!!

Yes, it's Princess Madeline's birthday. She is officially 11 years old. She's now 5' 1" tall and wearing a size 8 shoe. She's only two inches away from me! Wow...why do they have to grow up?

Like each year, I brought cupcakes for her class. Everyone sang happy birthday to her and she blushed, of course. This evening Madeline wanted to go out to eat at Texas Roadhouse. We surprised her with a Lulu (her toy poodle) cake. The staff came out screaming and yelling to everyone in the restaurant that it was Madeline's birthday. Then, everyone sang happy birthday to her. She cried. I didn't expect that. I then cried. My sweet, sweet girl. We surprised, no shocked her. But she loved it.

Each birthday I can't help but recall the first time I googled Madeline's disease. The first site I found was on a child with JDM being granted their "Make a Wish" gift. Of course, I was devastated and determined this was not our course (I'm somewhat of a control freak). Now, I am very optimistic and I KNOW she is going to be in remission one day soon. She WILL have a full life and achieve each and every dream she can fathom. And most of all...I know this little girl is going to be stronger due to this illness.

Today was a beautiful, crisp day....just like the day she was born 11 years ago. Today I celebrate the birth of my beautiful baby girl. But now, I also celebrate her life and the wonderful, happy, beautiful things that are to come to her in the future.

Happy Birthday, Madeline. Your daddy and I love you more than you'd ever know. We are so proud of you and honored God gave you to us to take care of forever and ever.

Sunday, January 10, 2010

Ouch...yucky throat!

We've had very unusual weather in our neck of the woods lately. It was in the 20s today and that doesn't happen here often. What is going on? We live in the south for a reason...mild winters, hot summers!!! I am so ready for a 100 degree heat index.

Madeline has earned herself a trip to the doctor. She has really looked tired and not herself lately. She told me today her throat hurt and that it felt tight. I took a peek in and let me just say if I was her I would be whining a lot more. It looks terrible. Keep your fingers crossed that it's something simple and a good old round of antibiotics will take care of.

It's methotraxate night. We've moved her once a week dose to Sunday nights now. Nothing like starting off the week with a little chemotherapy. :)

Friday, January 8, 2010

Two thumbs up on great research - release from Mayo Clinic

Any time I hear great news of JDM research...I love to share. This is not only good news for JDM patients, but potential all autoimmune patients. I don't know you...but great job, Dr. Reed! And thank you for your work!!!

Mayo Clinic provides new strategy for designing better treatments for juvenile dermatomyositis
Mayo Clinic researchers, working with colleagues at the University of Minnesota and University of Pittsburgh, are the in the first place to style a new role for a specialized apartment of the immune system in children suffering from a rare muscle-damaging disease known as juvenile dermatomyositis (JDM).

The specialized cells, called dendritic cells, have never before been found inside muscle tissue of JDM patients — a discovery that suggests they are tightly linked to initiation of the disease process. The finding opens new possibilities for designing better treatments for JDM, and possibly for other related diseases such as multiple sclerosis, rheumatoid arthritis and lupus.
The Mayo Clinic-led research team report will be presented Nov. 14 as part of the American College of Rheumatology’s annual meeting in San Diego, Calif., held Nov. 12-17.

Mayo Clinic researchers compared samples of muscle tissue from children with JDM to children with other disorders. Their findings are important not only for determining what causes JDM and designing new treatments for it, but for understanding an entire class of diseases in which the body’s immune system gets mixed up and attacks “self” as if it were a foreigner, or “nonself.” These are known as autoimmune diseases, and there are about 80 distinct autoimmune disorders. As a group, they are relatively common and include rheumatoid arthritis, lupus and multiple sclerosis. Autoimmune disorders share the general trait of the body failing to recognize itself, and erroneously mounting an immune attack that destroys function. Insights gained in JDM may possibly be applied to other autoimmune diseases.

Explains Ann Reed, M.D., Mayo Clinic pediatric rheumatologist/immunologist who led the investigation: “Under the microscope, it looked so dramatic to see the dendritic cells maturing in the muscle tissue and then migrating out into the bloodstream — and to realize it was a process which no one has ever documented before. And it was a surprise. Usually few dendritic cells reside in muscle as immature cells; they sort of hang out in case they’re needed in an immune response. But, we determined that they are actually maturing in the muscle tissues in response to something in the muscle tissue itself.”

This finding is important because a central question in JDM research has always been: Do the dendritic cells get activated in muscle tissue? Or, do they get activated outside of the tissue? The research by Mayo Clinic and collaborators provides the first proof that the dendritic cells get activated inside muscle tissues and then may move out into the bloodstream. Says Dr. Reed: “When you think about it as a clinician, it’s really exciting because it shows what is happening in the muscle that starts the disease — and holds out the possibility that it is maybe something that we can turn off in new treatments we develop by targeting the mechanism in the muscle tissue. And that’s really neat stuff for our patients.”

JDM is a rare (5 in 1 million children) autoimmune disorder of young children characterized by inflammation of the blood vessels under the muscle and skin. This results in muscle damage, as well as in tissue changes of skin over the eyelids, finger joints and knuckles. Symptoms appear gradually and include: muscle pain and tenderness; difficulty swallowing, which results in weight loss; irritability; fatigue; fever; and rash around the eyelids, finger joints, knuckles, elbows, ankles or knees.

Diagnosis may involve the following: blood tests to detect muscle enzymes and markers of inflammation; an electromyography (EMG) to assess nerve or muscle damage; muscle biopsy for examination; X-rays; and MRI. While there is no cure for JDM, there are treatment options. They include medications to reduce inflammation and skin rashes; physical and occupational therapy to improve muscle function; and nutritional support. Children with JDM may suffer organ failure in the same way transplant patients often do when their bodies fail to accept donated organs in graft-versus-host disease.

Monday, January 4, 2010

Blood work updates

I just picked up Madeline's blood work results and they all look great. Here they are:

SGOT (AST) 25 (0-31, normal range)
SGPT (ALT) - 21 (0-31, normal range)
CPK (CK) - 98 (24-170, normal range)
LDH - 187 (120-300, normal range)
Aldolase - 6.3 (3.3-9.7, normal range)

Woo-Hoo! Love it. What a great way to kick-off 2010!!!

New Years Eve 2007

New Years Eve 2007
Her rash on New Year Eve 2007. We were treating at this time as poison ivy.

Madeline, January 2008

Madeline, January 2008
Here is a picture of Madeline (2008) at her classroom birthday party. Here you will notice her malar rash and grottron's papules on her hands. Of course at this time we had no idea what was going on.

Madeline, January 2009

Madeline, January 2009
Here is a picture of Madeline at her 9th birthday party. The only redness you see now is the nornal redness from running around at your birthday party. My, what a difference a year makes.