Event and appointment back to back!
May 31, 2017
BENEFITING: Cure JM Foundation
EVENT DATE: Jun 04, 2017
We are Shawn and Liza Cobbs. Our daughter Maylea will be 8 this March.
Maylea was diagnosed in September 2014, at the age of 5 1/2, with JDM, Juvenile Dermatomyositis, the most common form of the Juvenile Myositis group of diseases. (For more about JM, scroll down or visit CureJm.org.)
Maylea's Path To Diganosis:
In December 2011, I took Maylea to the ER for unexplained swelling in her left knee. At the ER, X-rays were taken and Maylea was examined by the ER doctor as well as a Pediatric Orthopedic Surgeon. There was no diagnosis made and we were sent home being told to ice it and come back if it got worse.
For two and a half years the inflammation fluctuated in her knee. It never went away but didn't seem to bother her or slow her down. However, she complained periodically about leg pain and feeling as if she couldn't stand or walk, especially first thing in the morning and seemingly worsened by increased activity the day before. She would cry and tip-toe around not able to fully flatten her feet to the floor. I never connected the two, her unexplained knee swelling and the muscle pain and weakness. I simply brushed it off as "growing pains."
Then in June of 2014, Maylea complained for the first time of pain in her left knee. As it had for over two years, the knee appeared swollen and was warm to the touch. I did not heistate to take her to her pediatrician and explained the ER visit from three years ago. She ordered X-rays and found nothing. She referred us to a Pediatric Orthopedic Surgeon at Walter Reed National Military Medical Center. He ordered blood work and upon initial examination could not determine the cause of the swelling either. He referred us to Dr. Jones, a Pediatric Rheumatologist, also at Walter Reed.
In August 2014, we saw Dr. Jones for the first time and she ordered additional bloodwork and initially diagnosed her with Juvenile Arthritis and scheduled her for injections in the affected knee. She mentioned sunscreen and commented on her fair complexion.
After receiving the lab results Dr. Jones noted the increased muscle enzymes associated only with JM and after a second exam when she commented that the "rosie glow" on Maylea's cheeks (which I had always associated with a healthy glow under fair complexion), was actually a form of heliotrope rash, she changed the diagnosis to JDM and ordered an MRI to confirm.
October 2014, the MRI showed localized patches of inflammation in her thigh muscles as well as mysterious fluid in one hip and one knee and unexplained inflamation in her pelvic floor. All the pieces fell into place for the diagnosis, although questions still remain.
It took 34 months to get a diagnosis. That's over 1,000 days; more than 24,400 hours.
Maylea's Treatment & Prognosis:
Upon diagnosis Maylea was prescribed several medications: Plaquenil that we crush and give in applesauce every night, Folic acid that luckily has no taste and is given every night except Methotrexate days, and Methotrexate, a chemotherapy drug, given by injection once a week. As her parents, we are charged with giving the subcutaneous injections in her thigh once a week. Needless to say this particular aspect of the disease has been especially unpleassent for all involved. And to make matters worse Methotrexate causes nausea and vomiting for about 18-24 hours after injection.
We are fortunate however, that Maylea's prognosis is good. Her disease has not flared and although she is not yet in remission, we remain hopeful that the course will remain mild.
Her Treatment Plan:
Maylea must get routine blood and urine labs every two months to monitor muscle enzymes as well as kidney and liver function since the medications used to treat JDM can have harsh effects on internal organs.
Every 8-12 months she repeats the MRI to monitor muscle inflamation and every 6-12 months she has to have an eye exam to check for damage from long term Plaquenil use and/or inflamation in the eye. We also have physical exams every 2-3 months with Dr. Jones to make detailed observations on her strength and skin rash.
The "derma-" component of Dermatomyositis, means that Maylea's skin can be very sensitive to the sun, which is increased by the Plaquenil, and if she gets too much sun, it can trigger a flare in her disease possibly cauasing her to become sick enough to require hospitalization. Therefore, sun exposre is seriously limited especially during the peak hours of 10am - 4pm. High SPF sunscreen must be worn every day, even on rainy days, and sun protective clothing and hats are required for her to be outside.
Why We Are Raising Money For CureJm.org:
Cure JM's mission is to provide support for families coping with JM, raise awareness of JM, and fund research that will ultimately lead to a cure. Part of this mission includes working to significantly decrease the time it takes to receive an acurate diagnosis thus starting effective treatment as quickly as possible by developing educational materials and raising awareness within the medical community.
What Is JM/JDM?
Juvenile Myositis (JM) -- including Juvenile Dermatomyosits (JDM), Juvenile Polymyositis (JPM) and Juvenile AmyopathicDermatomyositis -- is a group of rare and life-threatening autoimmune conditions that affects 2 to 4 in a million children in the United States. Nearly 1,000 new cases are diagnosed annually.
JM attacks children of all ages, causing a variety of debilitating symptoms, including –
- muscle weakness and pain
- severe fatigue
- swallowing and digestive difficulties
- lung and heart problems
- vasculitic ulcers
Some children may experience remission, while others will battle JM their entire lives and complications from this disease can prove fatal. Medications can alleviate symptoms of JM, but there is no known cure.
Maylea and other children who are afffected by JM would greatly appreciate any help you could give.
The Cure JM Foundation is a volunteer-managed 501(c)3 non-profit organization in the USA.