Why not at the beginning of this weblog journey of my daughter, Cara's plight, from fancy, to fear, when we learned she was waging a fierce battle with a previously unknown to us, incurable and relentless, destructive childhood disease... Dermatomyositis.
Most of you who are now reading this may have already received a mailing or e-mailing or two which we sent out when all of this started. So some of this may be news you already know.
But for others, with whom we have had occasion to communicate in the other parts of our daily lives, much of this will be very informative and fill out some of the gaps in their knowledge and understanding of what Cara's disease is and its powerful potential to devastate her life... if it not leashed and restrained and put at bay. Through what we are now learning, we, ourselves, are finding that there are various workable and effective drug and treatment methods, along with some methods that, in trial, or in error, may or may not have worked as well as we would have hoped.
For this condition is a work in progress, and how it afflicts different children varies like night and day. But its continuing attacks on these growing and maturing young children, and as in Cara's case, whether mild or devastating, don't magically go away without a fight. And so it is.
So I will take you back to where it all began for Cara and us.
Cara was diagnosed in July 2007 with Juvenile Dermatomyositis; an extremely rare auto-immune disease with only about 5,000 known cases in the United States. This disease attacks the skin and muscles, is extremely painful and causes extreme fatigue, among a host of other things. There is no known cure, so for now it is generally treated with steroids and chemotherapy. The effect of this disease on its victims result in about a third going into permanent remission, another third having temporary remission periods, and for the remaining one third… it is a day-to-day battle for as long as they live. Tragically, in about one out of 100 cases, it results in death from complications of the disease. We have no way of knowing our Cara's odds of survival or health maintenance, but we live in hopes of an eventual cure, so far, undiscovered.
Before Cara was diagnosed, she was a level four gymnast and was in the gym at least three days a week. She also was a straight-A student, and she loved swimming, horseback riding, and going to the beach. She was a model for local department store flyers. She did television infomercials and print ads in her spare time.
Since then she has not been able to do any of these things as they have become too physically taxing for her. The beach and pool fun is now off limits, as one of the things that are harmful to children with this disease is sunlight and ultraviolet rays. Her life has changed dramatically for the worse. But she still maintains her courage, positiveness (most of the time) and straight A's somehow, even with all of the school she has missed. So far we have not yet come to the place where she may have to have home schooling, as some of the childhood victims of this disease who can no longer take the rigors of school attendance, must do.
SOME MEDICAL FACTS ABOUT DERMATOMYOSITIS
Also known as JDM, it is an autoimmune disease affecting approximately 5,000 children in the United States. Weak muscles and skin rash are the primary symptoms of JDM. Some children experience a mild form of the disease, while others follow a more severe and potentially more debilitating course. Some of the more onerous secondary symptoms are calcinosis, vasculitic ulcers and contractures.
In JDM, once a child's immune system turns on her infection-fighting process, she cannot turn it off. This process therefore damages the body instead of protecting it. One way the immune system cells fight infection is through inflammation. But when the cells cannot turn off the inflammation process, tissues are damaged. In JDM sufferers, the skin rash and weak muscles are caused by vasculitis, an inflammation in the blood vessels that lie under the skin and in the muscles. Since blood vessels run throughout the human body, JDM can also affect other systems such as the digestive tract.
Because this disease is so rare, it took us about three months to get Cara correctly diagnosed. There is currently research being done on this disease, however, there is no cure for JDM. There are only treatment options to help manage the symptoms. Early and aggressive treatment is usually the best predictor of a better outcome of this disease.
IV corticosteroids (Solumedrol) are usually the first line of treatment for JDM. This is oftentimes coupled with high dose oral Prednisone, another corticosteroid. Since the side effects of corticosteroids can be very troublesome, Methotrexate (a chemotherapy drug when given in higher doses) is usually introduced early to allow for tapering of the corticosteroids.
Other common treatments include Cyclosporine and Intravenous Immunoglobulin (IVIG). Less common treatments, but still used include Cellcept (chemotherapy), Enbrel and Remicade. These medications all have their own side effects, but the most common ones for Prednisone are: increased appetite and weight gain - rounded face - mood changes - high blood pressure - stretch marks - fragile bones and bone damage - cataracts - slow growth.
To learn more about this disease, please check out the website for the Cure JM Foundation... http://www.curejm.com/. There you will find Cara, among so many others like her, who have, for yet unknown reasons and by no fault of their own, contracted this disease. If you go to the "Faces of CureJM" on the Home Page, you will see these children, and their stories, as well.
From outward denial to reluctant acceptance, Cara’s health care odyssey has unfolded slowly, not yet unveiling significant clues as to where her journey is leading her.
OUR NEXT MEDICAL EFFORT
We took Cara to Chicago in April, May, July and October. Our next trip is this February 8th and 9th. She is seeing Lauren M. Pachman, M.D., Professor of Pediatrics, Northwestern University, Feinberg School of Medicine, and Director, Molecular & Cell Pathobiology at Childrens Memorial Research Center. Her credentials and medical expertise are top drawer. Cara has not only become part of the research that will help find a cure some day, but she is also getting the very best medical care that is available for her disease. Dr. Pachman has seen more cases of JDM than any other doctor. Families bring their children from all over the world to her for her expertise, and because she has seen the most cases, she has a much better overview of some of the mildest and worst cases of JDM.
She also has the very best laboratory equipment and researchers who are able to monitor the progress of her child patients. Cara had to have a mediport surgically implanted to enable infusion administrations and lab draws to prevent the risk of her veins collapsing due to these procedures. Cara is currently on twice a week Solumedrol infusions, which are administered at home by a home health nurse. She is also getting Methotrexate injections once a week, ably administered by her stepfather, along with oral Prednisone. She is taken by one of us, or other volunteering family members, to All Childrens Hospital in St. Petersburg once a month for IVIG (intravenous immune globulin) infusions which take all morning and afternoon. She also has out-patient physical therapy twice a week to try and rebuild her strength. These are followed up by three-times-a-day, at-home PT exercises. Also the wearing of lower limb and ankle braces through her nighttime sleeping hours. All of the medicines that she is taking have caused her to gain more than usual weight and have caused her face to become puffy-looking. Her rash is still very prominent on her face. Cara has had a truly emotional challenge dealing with her appearance changing. We have seen some improvement over the course of this treatment which we started in April, and Dr. Pachman feels that we need to continue with the treatment plan we are currently on for at the very least another 10 weeks. She has been slowly regaining her strength over the summer by swimming and doing exercises at home.
SOME WORDS FROM CARA
" I love Dr. Pachman because she knows so much about my disease and has helped me so much. I never thought that my family would be able to afford all of this, but I am thankful for all of the people who care about me and want me to get better, and have helped us. If you would like to send me a note or card or anything else like that, you can mail it to me, Cara Bodziak, 3935 Longhorn Drive, Sarasota, Florida 34233. And you could also email me at firstname.lastname@example.org. I would love to hear from you.
As always, love to you all,
THE LEARNING CURVE
We know from the histories of cases similar to Cara’s that getting her health back to what most of us would consider “normal good health” has taken in the neighborhood of five years, more or less. That is if the treatments prove effective, and other complications do not spring forth from the disease. In our Chicago medical trips we meet and get to talk to other families with their stricken children, who, like Cara, are in various stages of the disease. Some fare better, and some worse, but all, like Cara, have the best hope of recovery by some level of remission, and it does happen. If you have gone on the CureJM website, like most of us have, http://www.curejm.com/ and have checked out the publications, fliers and newsletters there, you have seen the best and the worst of worlds in the Dermatomyositis universe. So there is our fear, as well as there is our eternal hope.
So as we go along, we will do our best to keep you current with how Cara is progressing.
So as we go along, we will do our best to keep you current with how Cara is progressing.
I will do my best to keep you all up to date on Cara's progress, hopefully toward getting better, in future postings, about once a month. That will of course include the latest medical information we will have after returning from our February visit to Chicago.
So please check back in.
From Cara's mom, Krissy, God Bless and stay well.