Madeline's JDM Story: November 2009

Thursday, November 26, 2009

Happy Thanksgiving!

This Thanksgiving I am so grateful for…

being able to share our family’s story. I hope our story shared online with others is a source for you to grow hope, optimism and peace knowing there can be good outcomes with this disease.

I am so grateful for…

my wonderful and supportive husband who was especially strong at the beginning of our daughter’s diagnosis. He held me up for those first three to four months when I was living in a fog of depression and doubt.

I am so grateful for…

the talented and skillful physicians who diagnosed Madeline so quickly and developed a treatment plan that worked beautifully.

I am so grateful for…

being a mother to two incredible, beautiful, happy children who are growing up way too fast.

I am so grateful for…

my parents and in-laws who love us unconditionally, as well as our sisters, brothers and extended family and friends.

I am so grateful for…

our wonderful news that Madeline is on the course to remission.

I am so grateful for…

having the capabilities to provide for her care and in our small way assist with her healing process.

I am so grateful for…

God believing in Rick and I as parents - especially for the fact that he chose us as parents for a child who is ill.

I am so grateful for…

now seeing life differently.

I am so grateful!

Hope you have a wonderful and blessed Thanksgiving!

Gobble! Gobble!

Tuesday, November 24, 2009

Oops...we've already messed up!

One - forgot to post pictures of Madeline and her brother from Halloween. Two - we already got out of the habit and forgot to give Madeline her methotraxate Saturday night. I woke up in a panic Sunday morning. I couldn't believe we forgot. I guess not having to take medicine every night, you get lazy. I guess being able to think this way (forgetful) is a good thing....but only as long as we remember to take the medicine quickly.

Thanksgiving is just in a few days. But first, we will celebrate the birthday of my sweet husband and the wonder daddy to my two babies. Happy Birthday, Rick! I love you...I love us...I love our wonderful family!

Friday, November 13, 2009

Still celebrating...

We are still celebrating and adjusting to Madeline not taking her nightly medication, the cyclosporin. It's actually very odd. I keep thinking I'm forgetting something and then realize, our routine of her taking her meds is no longer happening. And that's a great thing.

I wanted to share some other doc day pics of us celebrating while in Texas. One is she and her daddy waiting to see Dr. W and the other being a celebration dessert at Olive Garden after the appointment.

Madeline is doing fine except she has a nasty cough and congestion. I believe she just has a cold, but will continue to watch her. Another thing she has just began to complain about is the top of her foot. She has a knot on it, but does not recall hitting it. We'll have to keep watching...and not thinking it's other things caused by JDM.

Breathe...relax. :)

Friday, November 6, 2009

And the results are....drum roll please!!!!

Does her face say it all??? Madeline's appointment went great. The doctor took her completely off her cyclosporine. This made her so excited. She hates taking that medication which is mixed w/ orange juice. It tastes terrible, so she says and so I can smell.

He was so happy with her results, strength, activity level, etc. We go back in three months and if all is still good, he will begin to decrease her methotraxate.

Pinch me...is this for real??? We are on the road...that beautiful road of remission. Thank you God!

Monday, November 2, 2009

A Texas We Will Go...

We leave at 7 a.m. to head to Madeline's doctor appointment in Texas. It's amazing how easy these appointments have become. I can remember the nausea that would set in and anticipation that would worry me the entire drive to his office. Now, it's not too bad. Especially since Madeline is doing so well.

Madeline's doctor is a pediatric rheumatologist at Texas Children's Hospital. I've always referred to him on the blog as Dr. W. At our last visit, he decreased her cyclosporine to 1 cc vs. her former 2.5 cc. This decrease began in September. As of today's date, we see no change in Madeline. She is doing really well. She still takes her methotraxate, 10 mg, each Saturday.

It will be interesting to see what he thinks. I don't believe he will decrease her meds any more tomorrow and we are okay with that. Slow and steady...slow and steady.

Wish us luck and say a little prayer for Madeline. I'll give an update when we get back.

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

Thursday, November 26, 2009

Happy Thanksgiving!

Tuesday, November 24, 2009

Oops...we've already messed up!

Friday, November 13, 2009

Still celebrating...

Friday, November 6, 2009

And the results are....drum roll please!!!!

Monday, November 2, 2009

A Texas We Will Go...

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