Madeline's JDM Story: Came across this post today...breaks my heart...please VOTE!

Friday, August 20, 2010

Came across this post today...breaks my heart...please VOTE!

Another parent with a JDM child had this posted on their blog today. I wanted to share it as well for it's a good reminder about the seriousness of this disease - this disease that our baby girl has fought herself. Here it goes...

Cole Flack of Oregon received his diagnosis of juvenile dermatomyositis in February 2007, roughly four-and-a-half years after Thing 1 received hers.

In the years that followed, this teenager with a love of sports and being outdoors became too weak to even walk. Cole endured multiple abdominal surgeries to deal with gastrointestinal issues caused by the disease and its medicinal treatments. Yet as recently as the fall, he still managed a full course load at school and compiled a 4.0 GPA.

Earlier this month, Cole went into the hospital with pneumonia.

Yesterday, we received this e-mail written by his parents:

“Hello friends and family,
Cole went to be with the Lord at 7 p.m. tonight. It was very peaceful. Cole is free now to run ... jump ... play baseball ... everything a 15 year old boy should be able to do. …”

Cole is the third child with a form of juvenile myositis that our family has come to know since Thing 1’s diagnosis to die from this rare autoimmune disease or its complications.

Three.

When only three in a million children are diagnosed annually in the United States with this disease, that little number grows exponentially in your worried head every time your child coughs or sneezes or scraps a knee.

Please help prevent more deaths of children like Cole by continuing to vote every day this month to help Cure JM, the only national nonprofit dedicated to supporting children with juvenile myositis and their families, win a $250,000 Pepsi Refresh grant. Every penny of that grant is set to pay for research into finding the cause and cure of juvenile myositis, juvenile dermatomyositis and other forms of JM diseases.

Right now, we are No. 2 in the standings – we need to hold this position until Sept. 1 to win the grant money. The only way that can happen is with your help.

You can vote up to 3 TIMES, every day, during the month of August!
(1) Send a text vote: Text 100850 to Pepsi (73774) (standard text messaging rate apply)
(2) Use the Facebook app: http://bit.ly/CureJMonFB
(3) Vote directly from the Pepsi website site for our Cure JM and its affiliated causes at http://pep.si/CureJMKidstoWin4

1 comment:

Summer said...: This really hits home hard! I saw this on Ericka's blog (Gary) and I posted about it as well!!

I have everyone I know voting for Pepsi Refresh and about 2 dozen of my blogger friends have posted on it as well throughout the month!! I posted again about it today

My thoughts and prayers are with Cole's family and it is just so heartwrenching to read about this....

Summer; August 20, 2010 at 2:48 PM

Post a Comment

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

Friday, August 20, 2010

Came across this post today...breaks my heart...please VOTE!

1 comment:

About Me

Want to contact Madeline or her parents?

Want to learn more about JDM? Visit...

Look whose following Madeline's blog

Make a Donation Today to Cure JM

Blood Work Results

What is JDM?

Prayers

Visitors