Madeline's JDM Story: June 2009

Sunday, June 21, 2009

To the Mountains

We've left on day one of vacation - the Smoky Mountains. Madeline is feeling and looking great. We'll post pics and give updates on our vacation.

The last time we were here was two years ago...before Madeline was diagnosed. I got sick while on vacation, as well as Madeline. I often wonder if this is what triggered her JDM. But who knows???

No thoughts like that now...only good ones and good memories to make this week.

Friday, June 19, 2009

Great news - blood work

Great news regarding Madeline's blood work. Here are the results.

AST - 25 (0-31 normal)
ALT - 11 (0-31 normal)
CPK (CK) - 112 (24-170 normal)
LDH - 225 (120-300 normal)
Aldolase - 5.2 (3.3-9.7 normal)

I was so excited when I saw her results. I know the reality is he probably had her do these tests as a base point to follow when we begin to decrease in September. But at least I know the medicine is working great and her little body is not inflamed. I do wish he'd ordered an ANA. I'll have to remember to request that next time we see him.

Now, it's time to pack...VACATION!!!

Tuesday, June 16, 2009

Blood draw

Madeline is attending art camp this week. They are making stained glass and I can't wait to see all the beautiful pieces she is making. Yesterday, after camp her dad dropped her off to me at the hospital and we had her blood drawn. I'm anxious to find out the results, especially with the additional testing. I'll go down to medical records later today.

We are getting ready for vacation...and it can not be soon enough!

Wednesday, June 10, 2009

Quarterly Photo Update

Attached you will find new photos of Madeline's JDM affected areas. She is looking great and has somewhat of a tan. I promise we are wearing sunblock, it's just her complexion tans easily. Last year, we almost totally stayed out of the sun. She only played outside in the evenings. She couldn't handle the heat and it hurt her skin. This is no longer a problem. Great strides...

Wednesday, June 3, 2009

Dr. Visit in Texas

We traveled yesterday to see Dr. W in Texas. Overall we had a good visit. Here is an overview of her height/weight, etc.

Height - 5 foot - a very tall 10 year old. Three more inches and she passes me up.
Weight - 95 lbs. - her brother, who is 13, only weighs five pounds more.
Temp - 98.5
BP - 112/64
Heart Rate - 103

We started out the appointment with the regular questions of how she feels, how is she doing, etc. We discussed her only problems being the various illnesses and ear infections we constantly battle. He discussed her flu and other various items. He then viewed her skin and completed her muscle strength testing. She pasted the strength test with flying colors. He even commented how strong she is. Nothing wrong there!!! GREAT!

From our last appointment he mentioned decreasing her meds at this visit. This left us anxious and excited. I had finally convinced myself this was a good thing and then yesterday he decided to not decrease her meds. I was a little disappointed. He wants her to get through the summer and decrease her cyclosporine in more than half in September. We will go from 2.5 cc to 1 cc. We will not go back to see him until October so he can see how she reacts. We will not decrease the methotraxate yet.

He also added additional blood work for her every two months. Now the order includes ALT, AST, aldolase, CPK and LDH. I will bring her this week for those tests.

Overall, this was a great appointment and I feel we are so lucky to have such great outcomes so quickly. With the decrease of meds this fall, we are actually at the two year mark for her first "symptoms" of JDM. Hard to believe it's been this long, yet at times, it seems just like yesterday.

One fun part of the appointment....Madeline watches a show on TLC called Little People. She realized that the female - Dr. Jennifer - works at Dr. W's hospital, Texas Children's Hospital. She wanted to know if he knew her, which he did. She thought that was awesome. Too funny.

Monday, June 1, 2009

If it ain't broke...

We spent the entire weekend outside playing in the pool. It was great weather and we loved every moment of it. Madeline had her good friend, Madeline, over to play. We put sunblock on many times, but Madeline's face still got a little red. When she woke up Sunday, it was still red. I feel so guilty.

We go to see Dr. W in Texas tomorrow. Besides the red face, Madeline also has a tan. We've put sunblock on but she is still tanning. She is like her dad who can get very dark easily in the summer. If he says anything, I'll remind him (Dr. W) that he was the one who said to continue letting her be a child and do regular activities. Swimming during the day (especially w/ a pool in your backyard) is one of those things. :)

I anticipate Dr. W decreasing her meds tomorrow. One moment, I am so excited. The next, I am horrified. I guess I have the mentality 'if it ain't broke - don't fix it.' We'll see.

I'll update you on the visit tomorrow. Wish us luck!

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

Sunday, June 21, 2009

To the Mountains

Friday, June 19, 2009

Great news - blood work

Tuesday, June 16, 2009

Blood draw

Wednesday, June 10, 2009

Quarterly Photo Update

Wednesday, June 3, 2009

Dr. Visit in Texas

Monday, June 1, 2009

If it ain't broke...

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