Madeline's JDM Story: February 2009

Saturday, February 28, 2009

Great week!

Madeline is still having a great week. Danced three hours today and she does not even seem tired. Awesome! It's Saturday night, which means methotraxate and cyclosporine. We have another busy day tomorrow. Trey is in a travel baseball tournament in Natchitoches. Madeline stayed with her grandparents today, but she will have to come with us tomorrow. Sunblock, here we come!

Thursday, February 26, 2009

Finally over the cold

Madeline is feeling better. It took over three weeks for her cold to go away. Glad her little immune system got rid of it. I find it does take her longer to get over minor illnesses, but the important fact is she does get better. She is really looking good and energetic. Good week!

I've slacked on keeping up with the post. We've been out of town, doing tons of school projects, travel baseball and this week my husband's been out of town for business. Boy do I miss him...and his help around the house and with the kids. :)

Thursday, February 19, 2009

Worry is my middle name

We've had a busy week, but I am so glad that we now have the Mardi Gras break. The kids are glad too. I think they need a break just as much as mom and dad.

It's report card and parent teacher conference day...so hope to hear good news. I'm sure we will. We have good and pretty smart kids. After that, Trey has a Jr. High baseball game and Madeline dance. It never slows down.

Madeline's tingling in her feet still bothers her..and me. I went into her room last night and she was crying because her feet were tingling. Any time she gets really mad or upset (which she had just done), her feet bother her. This is driving me nuts. I saw online (I know, don't look online) today this may be a sign of diabetes. Could her medication cause diabetes???

Always thinking too much and worrying too much...otherwise, always being a parent.

Sunday, February 15, 2009

The Big Bad Sun

Madeline had a good weekend. Our Saturday night ritual of taking her methotraxate is almost non-eventful now. Early on, the day after she took her methotraxate she was very tired. It does not seem to faze her any longer. Hope I didn't just jinx that!

We are gearing up and researching where to take our vacation this summer. Normally we are very outdoor people and plan vacations that focus on sand, beach, rafting, etc. We are trying to do something new this year so we don't have to have concerns of too much sun for Madeline. We are debating going back to the mountains (which we loved) or going to Washington D.C. (which would be the first for the kids). I am concerned about all the walking in D.C. for Madeline for she still tires out. In fact, last night I dreamed Madeline got sunburned on vacation. We panicked so much we started going to emergency room to emergency room. But no one could help. I know, nuts!

Anyway, Mardi Gras season is upon us as well as spring. We had another warm week and the grass and flowers are peaking out. In fact, my crazy husband got so excited with the warm weather, he decided to jump in the pool for a swim. But he didn't swim for long.

I've enjoyed this stress-less winter months with lots of rainy days where we don't have to fret of too much sun. But that concern is quickly approaching us with warmer weather, longer days and a bright sun getting ready to warm us up down south.

Thursday, February 12, 2009

Lingering cold

Madeline's week is going well despite she still has this lingering cold. We all seem to have it though and with our weather being as nuts as it has been, how can you help it. Today it's in the 70's, but just last week it was in the 30's and 40's. We have a lot "sinus" issues in this part of the world.

If she does not improve by this weekend, we will make a visit to see our favorite ENT - Dr. W.

Monday, February 9, 2009

Dancing Queen

Madeline and her team did awesome at the Dance Magic Competition in Mobile this past weekend. Madeline is still battling her cold, but never complained at all. They won gold in both their tap and jazz routines. Go Madeline!

How thankful I am she can dance!

Thursday, February 5, 2009

Dr. B visit tomorrow

We're packing tonight to go to Mobile, Alabama for Madeline's dance competition. We're stopping on our way in Baton Rouge to see Dr. B. Dr. B, the dermatologist, is the doctor who diagnosed Madeline last March. We haven't seen him since March 28, 2008, so it will be good to give him an update. We're actually stopping on our way for Rick to see him for his "skin issues" (nothing serious). It will be great to thank him in person for I believe this doctor's attentiveness and knowledge diagnosed Madeline early allowing her to get help and treatment quickly.

What do you say to someone that wonderful? How do we thank him enough? We will try our best. And I will try my best not to cry. :)

Wednesday, February 4, 2009

Sniffles

Madeline has had a cold for around a week now. Doesn't seem to be bothering her too badly. She did have a bloody nose last night, but nothing earth shattering. I'll just have to keep watching her to make sure she does not get worse. We have a big weekend for her coming up - dance competition in Alabama. We want to make sure she is feeling her best.

Monday, February 2, 2009

She did it!

Well, we've created a monster. Madeline won the title of Little Miss Queen of Louisiana yesterday. She was SO excited. Not bad for only her second pageant. She's a natural that one. She did great and what a wonderful confidence booster.

Last night while Madeline took her bath, I took the time to really look at her skin. I noticed she is scarred on her elbows and spots of her hands. Her chest cleared fine, as well as her face, ears and knees. The sores she got on her hands are the worse scars, but nothing like the actual sores, which were horrible. Glad that's behind us and things are looking so much better.

Trey didn't place in the science fair - oh well. A doctor one day he will not be, so that's okay. We are proud of him none the less.

Oh - a funny. After Madeline won the pageant, we told him he'd have to call her your highness. He said..."more like your hiney-ness".

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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