Madeline's JDM Story: November 2010

Saturday, November 27, 2010

All better

Madeline is much better. She should be good to go back to school on Monday. Hard to believe our Thanksgiving break has come and gone so quickly. With Madeline being sick and my dad in the hospital, it hasn't been the best holiday. But, we did put up the Christmas decorations and it has lifted our spirits. Plus, it was Rick's birthday (the big 39) and we had fun celebrating with him.

We got some cool news yesterday. Our local paper plans to run Madeline's story as the cover story on Monday. Hope she doesn't begin to get the big head. Ha! Nah!

Monday, November 22, 2010

What in the world???

Madeline's dad and I went to the LSU game this weekend. Madeline decided to stay at home with her grandparents to visit with her cousin who was spending the night too. When we got home, Madeline had spots all over her. She had two or three on her face Thursday, but I didn't think anything of them. Well, now, they are everywhere. She was vaccinated for chicken pox as a baby, but they sure do look a lot like them. We are going to the doctor this afternoon. Will update after. Here is what they look like.

Saturday, November 20, 2010

Madeline's Article in The Advocate

http://www.2theadvocate.com/features/109114629.html

Check out Madeline's article which was in The Advocate yesterday. The actual newspaper had six pictures of her, but there is one picture on the online version. Thanks to the writer Robin Miller for doing a great job. Enjoy!

Tuesday, November 16, 2010

Madeline's paper

Madeline had a small project where she had to answer a question in her journal for English. I'm always surprised when she writes about her disease. I thought I'd share her journal entry. The question she had to answer was - What is the best birthday gift you could ever receive?

The best birthday gift I could ever receive is for my disease to go away. It would be the best birthday present because I've had my disease for over three years and I need a break from it. That would be the best birthday present in the world and no one could argue any differently. I can't wait until my birthday. I bet I will be in remission.

Love, love, love that girl!

Super Star

Madeline is still on a high from her LSU Golden Girl experience. In fact, she's getting a little media attention on the whole experience. A Baton Rouge television station did a story, as well as The Advocate, Baton Rouge newspaper. Once they come out, I'll post on the blog. Then, locally, our NBC affiliate and local newspaper are doing stories. Cool!

I'm glad the Golden Girls will get the recognition they deserve for doing such a great thing for Madeline. But, I also love the fact that this disease will get some much needed exposure too. Who knows, maybe someone will see the article and realize the symptoms are the same their child is experiencing. You never know!

Wednesday, November 10, 2010

Madeline's Best Day of Her Life

Madeline had her LSU Golden Girl day experience last weekend. It was an incredible day and we met some fantastic, kind people. Besides the wonderful Golden Girls making her day special, LSU beat Alabama!!! Here are the pictures. I think they tell the story of the day better than anything.

Click here to view these pictures larger

Friday, November 5, 2010

Tomorrow's the big day & Cure JM video

Madeline is on cloud nine and can't wait for her Golden Day tomorrow with the LSU Golden Girls. We packed last night and she picked out her outfit. The funny thing is she decided to wear yoga pants just in case they asked her to dance. Too cute! Promise we'll take loads of pictures and post them. I'm so happy for her. She is blessed and so are we.

Also, I was sent the latest Cure JM newsletter today and on it there was a great video for those just diagnosed. I recommend you watch it. I commend them for producing the video. I know I would of found it useful and comforting for us. Here it is....

Wednesday, November 3, 2010

Madeline was all the buzz for Halloween

Here's a picture of my sweet little bumble bee. I've also included a picture of she and her brother...he was Jigsaw from the movie saw. Ugh, I hate those movies. Enjoy!

Oh...and she is feeling better. Maybe just regular old aches and pains. Gosh, I hate my head goes straight to the worse scenerio. Thanks for the extra prayers.

Blood Work Results

May 25:SGOT (AST) 24 (0-31, normal range); SGPT (ALT) - 21 (0-31, normal range); CPK (CK) - 111 (24-170, normal range);LDH - 189 (120-300, normal range); Aldolase - 6.2 (3.3-9.7, normal range)
Feb. 26:SGOT (AST) 27 (0-31, normal range); SGPT (ALT) - 21 (0-31, normal range); CPK (CK) - 159 (24-170, normal range);LDH - 170 (120-300, normal range); Aldolase - 4.1 (3.3-9.7, normal range)
Dec. 31: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)
Aug. 27: ALT - 10 (normal range 0-31); AST - 22 (0-31, normal); CPK - 125 (24-170, normal); LDH - 211 (120-300, normal); Aldolase - 4.6 (3.3-9.7, normal)
June 15: ALT - 11 (normal range 0 -31); AST - 25 (normal range, 0-31); CPK - 112 (normal range, 24-170); LDH - 225 (normal range, 120-300); Aldolase - 5.2 (normal range 3.3- 9.7)
March 20: ALT 11 (normal range 0 - 31); AST 24 (normal range 0 - 31)
Dec. 29 - ALT 10 (normal range 0 - 31); AST 20 (normal range 0 - 31)
Oct. 24 - ALT 10 (normal range 0 - 31); AST 24 (normal range 0 - 31)
Aug. 22 - Cyclsporine A Serum leve: 97.5; ALT 9 (normal range 0-31); AST 22 (normal range 0 - 31); urinalysis - all clear
July 14 - Cyclosporine A Serum level: 141.0
June 27: ALT 11 (normal range 0 - 31); AST 24 (normal range 0 - 31)

What is JDM?

Source curejm.com
Juvenile Myositis (JM), including Juvenile Dermatomyositis (JDM), Amyopathic Dermatomyositis (AMD) and Juvenile Polymyositis (JPM), is an autoimmune disease affecting approximately 5,000 children in the United States. Weak muscles and skin rash are the primary symptoms of JDM, while muscle weakness without a rash is the primary symptom of JPM. Children afflicted with AMD, on the other hand, experience the skin rash without the weakened muscles. Even within these three designations, JM affects every child differently. 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.

Genesis of the Disease
The immune system is a group of cells that normally protects the body from infection. In autoimmune diseases like JM, however, once these cells turn on their infection-fighting process, they 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 JM 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, JM can also affect other systems such as the digestive tract.

What causes JM?
Many researchers believe that there is a genetic predisposition to autoimmune diseases. As such, they feel that a child afflicted with JM is likely to have a blood relative who suffers from another autoimmune disease such as diabetes or arthritis.

The autoimmune disease initially presents itself when an invasive "trigger" causes the body's immune system to overreact. It is believed that this trigger could be a virus, a vaccine or an environmental hazard.

What are the symptoms of JM?
The primary symptoms of JM are weak or painful muscles, skin rash (with JDM), fatigue and fever. Some children experience joint pain as well.

Weak muscles
The muscles that are affected the most are near the trunk of the body: the stomach, quadriceps, neck and biceps. However, other muscles can become weak as well. Sometimes there is inflammation in the esophagus which makes it difficult for the child to swallow. Other times, there is inflammation in the gastrointestinal tract, which causes bowel difficulties.
A child with JM has great difficulty climbing stairs and getting up from the floor or from a chair. Walking and running become very challenging and exhausting.
Skin rash
The skin rash in JDM usually occurs on the face, knuckles, elbows, knees and ankles. The rash may appear before, after or at the same time as the muscle weakness. Sometimes the rash is so faint that it is not noticeable.
The rash appears as a red purplish color on the eyelids and cheeks; whereas the rash looks like red dry skin patches over the knuckles, elbows and knees. The fingernails and the nailbeds may take on a pinkish color as well.
Sometimes, the children develop Gottron’s Papules, which are small whitish bumps that appear on the fingers. They look like small blisters.
Fatigue
With JM, the child becomes tired easily and can only walk short distances. The child needs to rest often and lacks the energy for normal activities. It becomes difficult for the child to keep up with friends.
Fever
Oftentimes, the child with JM runs low-grade fevers, especially at night. There has also been a connection discovered between a virus with high fevers just before the onset of JM.

What are other possible symptoms?
The following symptoms of JM are less common, but important to understand:

Calcinosis
About a third of the children with JM will develop calcinosis, which are small lumps of calcium that form under the skin or in the muscle. They may feel like little rocks under the skin and can range in size from a dot to a large pebble.
The lumps can either break through the skin and form a bump that may leak creamy white calcium, or they can be absorbed back into the body. If they break the skin, they can become infected and painful, and sometimes need to be surgically removed. They usually occur on the joints, such as the elbows and knees, but they can occur anywhere on the body.
Vasculitic Ulcers
Vasculitic ulcers are holes in the tissue that surrounds an inflamed blood vessel. They can occur in the skin or in the bowels (the organs that transport food from the esophagus to the rectum).
When they occur in the skin, they look like sores in the skin rash. They can be very deep or on top of the skin and they also range in size. The ulcers can be very painful, but will usually go away with treatment.
When the ulcers occur in the bowels, it is due to inflamed blood vessels in the protective lining around the bowels. They cause a break in the lining which can eventually harm the digestive organs.
The warning signs of this condition are severe stomach pain, dark black stools or blood in the stools, or a change in the pattern of bowel movements.
Contractures
Contractures are shortened muscles that cause a joint to stay in a bent position. These can occur due to lack of exercise or from scarring of the muscles.

What tests are used to diagnose JM?
If JM is suspected, blood tests are the first step to confirm that there is a problem. Muscle enzymes are measured, including CPK and aldolase, which are enzymes that leak from inflamed or damaged muscles into the bloodstream. Antinuclear antibodies (ANA) are also measured to see if the child’s body is producing antibodies against its own cells.

The next step is usually an MRI which can also detect muscle damage and inflammation. If the MRI shows evidence of diseased muscles, a muscle biopsy is typically performed to finalize the diagnosis. A small amount of muscle is removed for examination to determine if and how much the muscles and blood vessels have been affected by the disease.

What is the treatment for JM?
There is no cure for JM yet, but there are 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 JM. 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

What is the prognosis?
It is believed that a third of the children will suffer from JM most of their life, while another third will experience temporary remissions and the other third will experience a permanent remission (no medications, no symptoms).

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