Madeline's JDM Story: October 2009

Thursday, October 29, 2009

Punk Rock Girl

We are ready for Halloween. Madeline (after changing her mind around 10 times) finally decided she is going to be a punk rock girl. Wasn't real sure what she was thinking, but once we bought everything and she tried her outfit on I realized she's not a punk rock girl but an 80s girl. She is wearing almost everything I did in 7th and 8th grade...kind of an Madonna look when she first started out with Lucky Star. Too funny.

We are planning our annual Halloween get together so the house will be packed. Speaking of house packed...we have new live-ins - my in-laws. We moved them in this past week while their new home is being built. Plan is they will be out before Christmas. Thanks goodness I love these people.

Thursday, October 22, 2009

Journal of Pediatrics on JDM

I'm always interested on any study conducted on JDM. Found this one which would include Madeline in the mix since she never took corticosteroids. Interesting...

Favorable Outcome of Juvenile Dermatomyositis Treated without Systemic Corticosteroids
Deborah M. Levy, MD, MSa , C. April Bingham, MDb, Philip J. Kahn, MDc, Andrew H. Eichenfield, MDa, Lisa F. Imundo, MDa
Received 2 March 2009; received in revised form 6 August 2009; accepted 2 September 2009. published online 21 October 2009. Corrected Proof

Objective
To describe the course of patients with juvenile dermatomyositis (JDM) treated effectively without systemic corticosteroids.
Study design
A retrospective study of 38 patients with JDM treated at a tertiary care children's hospital identified 8 patients who had never received corticosteroids. Disease presentation and course, pharmacologic, and ancillary treatments were recorded.
Results
Patients in the no corticosteroid group were followed for a median of 2.8 years (range, 2.1 to 9.5 years). Treatment was primarily with intravenous immunoglobulin (IVIG) (75%) and methotrexate (50%), with favorable response in all. No serious treatment complications were observed; headaches were reported by 3 patients receiving IVIG. Two patients had a myositis flare after discontinuing all medications for more than 1 year; complete resolution of symptoms was observed after either 1 or 2 further doses of IVIG. Two patients had calcinosis (at 1 and 9 years of disease); however, no patient had joint contractures, muscle atrophy, lipodystrophy, or functional limitations.
Conclusions
Systemic corticosteroids can be avoided in a select group of patients with JDM. Alternative agents such as methotrexate and IVIG may be prescribed to effectively treat JDM and prevent complications.
ALT, Alanine aminotransferase, ANA, Antinuclear antibodies, AST, Aspartate aminotransferase, BMI, Body mass index, CMAS, Childhood Myositis Assessment Scale, CPK, Creatine phosphokinase, CUMC, Columbia University Medical Center, DsDNA, Double-stranded DNA, ENA, Extractable nuclear antigens, IVIG, Intravenous immunoglobulin, JDM, Juvenile dermatomyositis, LDH, Lactose dehydrogenase, MMT, Manual muscle testing, MTX, Methotrexate
a Department of Pediatrics, Morgan Stanley Children's Hospital of New York-Presbyterian, Columbia University Medical Center, New York, NY
b Department of Pediatrics, Milton S. Hershey Medical Center, Penn State College of Medicine, College Station, PA
c Department of Pediatrics, New York University School of Medicine, New York, NY

Sunday, October 18, 2009

Fall has finally arrived

After a week of 90 degree temperature, along with humidity of 100 percent, the cool air has finally arrived to Louisiana. It's a beautiful weekend with temps in the 60s-70s. Madeline is enjoying the day by playing outside and riding her bike with friends. Love it!

Madeline's decrease of meds is still on course. I've not seen one effect due to the decrease. I don't want to jinx us, but I really think she has beat this stuff. It has been a year and seven months...I think that's long enough. Oh - I hope we're this lucky.

Wednesday, October 14, 2009

2009 5th grade school picture

This is the official photo of Madeline's 5th grade school year. My, my...she looks way older than 10 years old. But, she is beautiful and looks, feels wonderful. God is good!

Friday, October 9, 2009

Geaux Tigers!!!

We are heading out today for the LSU/Florida game. We are so excited! Everyone tells us it's nuts in Baton Rouge already. We plan on dressing the kids up and bringing them to ESPN Gameday. Hopefully, we'll get on TV...watch for us!!!

Geaux Tigers!!!

Monday, October 5, 2009

Madeline's weekend

We had a nice weekend. Our son Trey went out of town for a birthday party so we had Madeline all to ourselves. We let her select what to do so off we went to Copeland's and then the Halloween store. We had fun except for the part that the Halloween costumes all looked like stripper attire. We decided we could find a better costume somewhere else and pushed her out of the door quickly.

Madeline went to a cheer camp at a local school Saturday morning and had a great time. She was as tall and talented as most of the cheerleaders there. But I guess I am biased. :) After, we watched a very incredible, yet nerve wrecking LSU/Georgia football game. Our Tigers WON!!!!!!

Great, calm weekend. It rained, was relaxing and enjoyable. We needed this simple time. Oh- her Texas doc appointment can't happen until early November. No big deal though for all is good.

Thursday, October 1, 2009

All is good

Madeline continues to do well. I was so concerned that she would return to school and be out again with another illness. Luckily, this has not been an issue.

We are calling today to schedule her check-up w/ Dr. W in Texas. This is the longest (3 months) we've gone without going for a check-up. This was because he wanted to give her a chance with the decrease of cyclosporine. He wanted to see if we experience any changes or flares w/ her JDM due to the decrease. So far - we see nothing.

I know it's way too soon to get too excited, but at least it's good news for now. I believe he is going to be very pleased with her results. And of course, I then wonder when we will be able to decrease other drugs and be on our way to the big R...remission!

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, October 29, 2009

Punk Rock Girl

Thursday, October 22, 2009

Journal of Pediatrics on JDM

Sunday, October 18, 2009

Fall has finally arrived

Wednesday, October 14, 2009

2009 5th grade school picture

Friday, October 9, 2009

Geaux Tigers!!!

Monday, October 5, 2009

Madeline's weekend

Thursday, October 1, 2009

All is good

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