Madeline's JDM Story: September 2009

Friday, September 25, 2009

Update on Princess Madeline

I am terrible. I've dropped the ball and not updated Madeline's blog. So many of you have sent notes worrying about her. Thanks for caring.

She is better. It took 10 days (9 days straight w/ fever) to get over the flu. Her rash is healing. The pits have healed nicely, just hope there are no scars. I do not believe the rash is JDM related, but due to the illness. Now, don't get me wrong. If my healthy son had the same illness, I do not believe he would had reacted like she did with the longevity or rash. But who knows. I'm just glad she is back at school...which she missed way too much of...back at dance....which she missed over two weeks worth...and back to being a happy, well little 10 year old.

This illness scared Rick and I. I've never had either of my children sick for this long. Even my son who had meningitis got over the illness faster than this.

But all is now good in Madeline's world today. She's well, dancing and playing w/ friends. And, she got her first report card for the school year and almost made straight A's. She missed it by one point! But we are still SO proud of her. What an amazing little princess.

Wednesday, September 16, 2009

Day 6 and we still have fever!

I am almost in panic mode. Madeline has now had fever for 6 straight days. I've decided that if she still has fever tomorrow (after also taking a z-pack) I'm calling our local pediatric infectious disease physician for an appointment. Our pediatrician is nice, but not as aggressive as I'd prefer. Plus, the doctors' offices are packed with kids with flu, mono, strep and that's that last place I want to bring her right now.

I'm trying to not worry, but I can not help it. She has never had a constant fever, that's very high at times, for this long. Neither of my kids have ever done this. She has now missed a week of school and I can't even imagine how she will get caught up. But then again, who cares. I just want her well.

She seems more congested today. Her nose, head is very stuffy. When she does not have fever, she's in good spirits. She freezes constantly though - with or without fever. And her arm and chest rash still looks bad.

I guess I am rambling...but I'm concerned.

Monday, September 14, 2009

Look at her arm!

Madeline is still very sick. She has a high fever and can't seem to get over this quick enough for Momma. One concern I have is the rash our doctor says is poison ivy. I'm not convinced. Here is a picture. It looks terrible.

Saturday, September 12, 2009

The nose swab - yuck!

Here is a pic of Madeline having her nose swabbed to identify the flu strain. She said, "it tickled very much but did not hurt at all." She is a little better today. Her fever is not as high. We've been inside all day and it's getting a little old.

Friday, September 11, 2009

The Flu is HERE!!!

We tried to protect Madeline as much as we could, but it didn't work - she has the flu. We went to the doctor today and they swabbed her. We still have to wait on the confirmation of the swine flu, which to me really doesn't matter because by the time we get the results hopefully she will be well. My understanding is they send the swab to Atlanta and it takes 3 -5 days for results.

She is experiencing the normal flu symptoms...sleeping a lot, fever, shakes, burning up, high fever, aches and headaches.

We had a weekend getaway planned to Baton Rouge. It's the first home game for LSU and we go to every game. Plus, we planned to do some girl shopping while there. The Tigers will have to win without us. But, we will be cheering for them from home.

GEAUX Tigers!!!

Thursday, September 10, 2009

Not feeling well

Madeline came home from school today not feeling well. She called me at work crying and weepy. She didn't go to dance tonight. She is not running fever, but her throat looks terrible. She is very tired and just not herself. We'll have to just wait and see.how the night goes.

I did notice a rash on her arm. My first reaction was a little panic, but the more and more I look at it I believe it's poison ivy...I think. She went camping with a friend last weekend. So, maybe that's what she has. She has never been allergic to it, so if it is poison ivy this is a new allergy for her. I will keep a close eye on it.

We are on day 10 of her decrease of meds. This couldn't be the JDM - could it?

Tuesday, September 8, 2009

Blood work results

Great news on blood work...see for yourself!

SGOT (AST) - 22 (0-31, normal)
SGPT (ALT) - 10 (0-31, normal)
CPK - 125 (24-170, normal)
LDH - 211 (120-300, normal)
Aldolase - 4.6 (3.3-9.7, normal)

Tuesday, September 1, 2009

First day of decrease of cyclosporine

Today was our first day of the "decrease". We decreased her cyclosporine, by doc orders, to 1 cc per day. When I made her medication, which she drinks w/ OJ, I didn't think about not putting as much OJ in the glass. She made an awful face. I asked her, "what is it"? She said, "I can taste how sour the orange juice is...it's terrible".

I've had a very busy week at the hospital and not had the chance to pick up her bloodwork results. Still have the fear of the flu in our community. Our local schools and hospitals have been hit hard. Today, while walking through our emergency department, there was a line out the door for people waiting to be triaged...all obvious flu-like symptom patients.

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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