Madeline's JDM Story: June 2010

Tuesday, June 29, 2010

Have you wondered where we were?

I know, I've not posted in a while. But, I have good reason - VACATION!!! We just got back from Florida....and yes, there was oil. But luckily not the entire time. The first half of the vacation the water was perfect, then, mid-way, the oil blew in with the bad weather. It was terrible.

Besides attaching a yuck picture of the oil, I'm also including a pic of Madeline. I know, she is beautiful!So was the beach and water, at first. :)

Wednesday, June 16, 2010

A star, or two, are born...

Everyone's a star and deserves the right to twinkle. - Marilyn Monroe

Madeline is still doing great. As we laid in bed last night watching The Incredibles, I asked her how she was feeling since stopping her meds. She stated, "Wonderful! I feel better than ever. That medicine made me feel sick. Now I don't feel sick anymore." That was music to my ears.

Madeline loves Marilyn Monroe - I guess because she is pretty, wears shiny things and a lot of make-up. I came across this quote the other day and couldn't help but think about Madeline. And how appropriate to share on the blog since Madeline and her brother are recently starring in a news spot for our local television station. Check it out. You'll see Madeline for she is the swimming girl and the trampoline jumper. Her brother Trey is cleaning the pool (which by the way he never really does) and in the fishing scene.

My two little stars...just a twinkling.

Friday, June 11, 2010

Beautiful Bloom

Madeline had to take a picture yesterday of her first sunflower she grew in her flower garden. She is so proud.

We are on week two with no meds and she is doing great. I don't know what to expect, but I can't stop worrying about the what ifs.

Monday, June 7, 2010

Madeline update

It's now day four with Madeline taking her medications for strep throat and tonsillitis. The drugs are working well. Literally, on day two her throat stopped hurting. She's back to normal, but still has to complete six more days on antibiotics.

We are still watching the oil situation in the gulf. We still plan to go to Florida even though tar balls have been spotted on the beaches. Around here, that's were everyone goes on vacation. But, so far, no one has told me anything too scary regarding the beach, smell or dead sea life on the Florida shores. Now here at home(Lousiana) that's a different story...and a sad one too.

Well, Sunday passed and went and no medications had to be taken for her JDM. Weird! But oh so nice!!!

Thursday, June 3, 2010

791 days...but who's counting???

First, the good news. Madeline's doctor appointment in Texas with Dr. W was great. All of her tests were perfect. She's strong as an ox. Her bloodwork was once again perfect. Dr. W was so pleased with Madeline's condition, he officially has taken her off all of her medications. Can you give me a woo hoo!!!!

We were so excited when he told her this fabulous news. It actually for once feels real. I see she is perfect, the tests tell us she is and the doctor has blessed it. Thank you God! We all hugged and teared up a little. After 791 days of taking medication to fight her JDM...we are done! Really, really done.

Now, the bad news. Madeline always wants to go to Olive Garden after her appointments. It's a tradition we do each time now. After being seated at our table and deciding what we would eat, Madeline stated she didn't feel well. She thought she was going to vomit. I wasn't real alarmed because this happens sometimes after taking her methotraxate. I thought once she ate she would feel better. After the food came, Madeline excused herself. After a few minutes, I went to check on her and the poor thing had vomited everywhere in the bathroom. The sweet employees were helping her. We left soon after and she slept our long drive home.

Later that evening, Madeline said she was freezing...um, the fever alert went off in my head. We took her temp and it was 102.9. Wow! And it only got higher as the night went on. But, she had no symptoms except for her vomiting (once). I felt it must be viral and the next 24 hours would be telling. Wednesday, her fever was still there and later in the day...her throat began to hurt. Aw, now we know the problem. We called the doctor, but could not get in until today.

Long story short, we saw our ENT this morning. The verdict...tonsillitis and strep throat. Really!?!? Wow...cher bebe. She is a sick little girl. So what happens now? We get off her JDM meds to only have three new medications to take. "That's rather ironic," said Madeline. Yes it is.

But, we are on the path to remission. We have a lot to celebrate and be thankful for. We do not have to go back to see Dr. W for six months...that's December 7. Of course, if anything changes we must call him immediatly. But my gut says it's going to be alright.

189 days until we see Dr. W again. That's 189 days until we can say Madeline is in complelty, absolutely, 100% in remission.

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).

Madeline's JDM Story

Madeline's Blog Archive

Tuesday, June 29, 2010

Have you wondered where we were?

Wednesday, June 16, 2010

A star, or two, are born...

Friday, June 11, 2010

Beautiful Bloom

Monday, June 7, 2010

Madeline update

Thursday, June 3, 2010

791 days...but who's counting???

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