Saturday, October 31, 2015

Happy Halloween

Happy Halloween 
From The Bug and Bubba Jay
Today is a pretty good day, feeling excited about truck or treating

Friday, October 30, 2015

Pics ....10/30/15



Here are the pics of Uncle Bubby and The Bug at the pumpkin patch this am...
Her little face has gotten so big .... She's gained 20 + lbs within the last month due to her medications.... She told her mom the other day when her jeans no longer fit her, "Mom, I'm getting so fat!" Kayla is a bit worried about her self esteem... We will continue to praise her braveness, strong will, determination and beauty!!!
She is an INSPIRATION!! 


Field trip with School

This morning Buggy had a field trip to Happy Jacks Pumpkin Farm.
It's been raining the past couple days and has turned cool.. So it's wet n chili...
But Kayla bundled her up and allowed her to attend the trip with her class.
Uncle Bubby went and met up with them at the farm to surprise her ( She's Bubby's girl) and to help take care of her if a need arises.
So far things are going well....
I hope to post pics later ....

Tuesday, October 27, 2015

Two steps forward three steps back

Sorry I'm just getting a chance to post this... I will try to do better at keeping everyone abreast at Taylor "Bug's" battle.

Kayla called this past Saturday, it seems perhaps Buggy is having a relapse... The rash on her face has returned as well as the blistery sores on her knuckles. She's extremely tired these days and struggling with walking for very long periods.

A call to her specialist yesterday, he wants to stop the weaning process of the steroids and go back to the more aggressive dosage. We are trying to keep from having to do another IVIG, or upping the dose of her chemo.
She is still being such a trooper!! We are so very proud of her. She is an inspiration to us all.

She has a field trip this Friday to the pumpkin patch, Kayla is very adamant that Taylor has as normal a childhood as possible. We are praying the stronger steroids will bring her strength up and she'll be able to enjoy the trip with her class mates.

Fundraiser NEWS-
Carl has hit the road running- he has had positive feedback and support from the local car dealerships ( Cars for a Cure) as well as local businesses. Please if you feel lead to give to CureJM Foundation... You may donate directly on their website.
It IS a Non Profit organization as well as tax deducted -95% of proceeds go to research. Please help us find a cure.
CureJM.org 
In the memo please add Taylor/Bug. So we can track the donations.
Thank you again for your continued prayers, love, support and kindness.

Love,
Bug

Monday, October 26, 2015

Awareness Monday from CureJM

#AWARENESS MONDAY! What is LIPODYSTROPHY? 
- Lipodystrophy is when inflammation from JDM affects the deeper fat tissue under the skin, destroying or damaging fat cells. 
- Lipodystrophy causes problems with the body's ability to use sugars & fats, leading to metabolic problem. Other issues could be insulin resistance, hyperlipidemia, and pancreatitis.
- A pediatric endocrinologist may be one of the specialists who helps to consult/treat Lipodystrophy, in addition to the pediatric rheumatologist. 

For more info, "Chap 26: Too Much Body Fat Loss" of "Myositis & You" Book covers Lipodystrophy! 

www.CureJM.org

Wednesday, October 21, 2015

Mom's Battle with the School

My mom sent a letter to school with me the day after I got out of the hospital. Explaining the disease I have and that I can't play outside for very long at recess with the rest of my friends. When I do go out I have to wear sunscreen and my floppy hat.
She also told them about my legs and muscles,that I would get tired easily and have trouble climbing stairs and in gym class.

It didn't help. I had gym and the teacher made me run... a lot.
When I got home my legs hurt so bad. I told my mom and she was really mad.

But it was time for fall break and I would be out of school for three weeks, so mom requested a meeting with the Principal and my teachers....

Fall break is over, my mom has a meeting Thursday, she's requesting a 504 plan.... This is suppose to help me, the teacher have to follow what it says.

I don't like being sick.

You can go to this link and it will help you understand what a 504 plan is and why I need one.

http://www.pcdfoundation.org/pcd-at-work-and-school/what-is-a-504-plan

Pumpkin Patch

Early Sunday Pops and Nana took Joe ( my brother) and Me to Happy Jack's Pumpkin farm. Even though the sun wasn't hot, I have to protect my skin, so I wear sun screen and my hat. I can only be outside for a short amount of time.

Feeding the Goats and Lamb....


I'm growing tall.
The steroids have made me gain weight. I told my mom I was getting fat, none of my clothes fit me any more.

Getting my face painted, I did a lady bug because my family calls me Buggy or Bug. Because when i was in my moms tummy she talked to me and called me her Tater Bug... 

We've been at the farm for fifteen minutes......I'm already getting tired...My legs are beginning to ache, but we are going to go on a hayride to the pumpkin patch.


video
Pops helped me get my pumpkin, after walking up and down the patch, my legs were hurting so bad, this is because the disease I have attacks the cells in my muscles. I'm one of the lucky ones... right now its attacking my tummy, hips, and leg muscles. Sometime it attacks kids hearts, and other vital organs.










Bug’s Battle

Friday, October 9, 2015

Bug's Out- Patient IVIG Treatment

Today is October 9, 2015.
Mom and Bug were up early this morning for an hour long drive in early morning traffic for Bug's first out patient IVIG treatment....

You may be asking what is IVIG?  How is it suppose to help?

Let me answer those questions for you.

IVIG stands for Intravenous Immunoglobulin
Intravenous- IV
Immunoglobulins- are plasma proteins secreted by plasma cells, forming a major component of the adaptive immune system. 

**Immunosuppressive therapy has played a very important

role in reducing mortality rates and morbidity in JM patients.



IVIG is a blood product prepared from plasma, each batch prepared from a pool of 10 000–20 000 donations. Multiple purification steps during the manufacturing process aim to eliminate all known transmissible pathogens, but cannot completely exclude the risk from unknown pathogens.

** So you see not only does Bug and the other children effected with this disease have to deal with the effects AND complications of the disease itself, but also the possible effects of their treatment**

 MORE FACTS:

Intravenous Immune Globulin

– Immune Globulin – antibody, protein, Y-shaped,
protect from foreign invaders – most common is
IgG
– Found in blood plasma
• Human donors – it takes THOUSANDS to make just ONE DOSE
• Plasma is treated to deactivate any potential viral
contamination
• Plasma is purified so that contents are as much IgG as
possible and minimize other plasma contents

How is it administered?
• Usually through a vein in your arm
• If you have difficulty with IV access, central
line in chest is an option – more long term
• Slowly – most reactions occur when it is run
too quickly
– Infusion rates are specific to each individual
– Package insert is the maximum rates for anyone,
but side effects tend to decrease the more slowly
it is infused
.
How is the dose determined?
• By weight and diagnosis
EXAMPLE:
Someone with myositis is typically prescribed
2 grams per kilogram.
80 kg person = 160 grams
This would likely be broken up into 4 days of
40 gram each day
40 grams each day should take about 3-5 hours
depending on:
– MD orders
– Any prior treatment with IVIG and how it was
tolerated
– How the individual actually tolerates IVIG when
administered
– What the package insert for the brand used says

– Any rate restrictions for cardiac or renal issues

What are the side effects of IVIG?
Severe adverse reactions
• Anaphylaxis
• Renal Failure
• Thrombotic Event
• Aseptic Meningitis
Syndrome
• Hemolytic Anemia

Infusion-related reactions
• Headache
• Blood pressure changes
• Nausea
• Vomiting
• Dizziness
• Fever
• Fatigue
• Chills

How does it work?
• Not completely understood, there are many theories
• Myositis is thought to be autoimmune disease
– Autoimmune disease is where the immune
systems perceives part of the body as foreign and
attempts to destroy it
– IVIG suppresses the immune response
– IVIG has anti-inflammatory properties

Normally the drip of the IVIG is set for a 4 hour OR 8 hour session. 
Meaning it takes that long to be completely administered.
That"s a very LONG time for anyone, imagine for a 6 yr old. She has to stay still, hooked up to the pump as well as heart rate and pulse monitored.

Bug is a very DETERMINED, BRAVE, and STRONG little girl
As you can see in the pics.. At first they were using her right arm, however, she blew a vein and they had to move over to the left arm and start over.


http://www.myositis.org/storage/documents/2012_AC_Presentations/tma_2012-greer._ppt.pdf

Sunday, October 4, 2015

FACTS ABOUT JM

Juvenile Myositis (JM), including Juvenile Dermatomyositis (JDM) and Juvenile Polymyositis (JPM), is a group of rare and life-threatening autoimmune diseases, with these diseases the body’s immune system attacks its own cells and tissues.

Weak muscles and skin rash are the main symptoms of JDM, (What Taylor has) while muscle weakness without a rash is the main symptom of JPM.

JM is a rare disease, what causes this disease is unknown. Approximately 2 to 4 children in a million in the United States are diagnosed with JM each year. That is 2-4 TOO MANY!!!
JM begins in childhood or the teen years. For some reason the average age a child is at the time of diagnoses of JDM is between six to seven years old; 25% are age 4 or less. JPM usually develops several years later.
JM affects girls twice as much as boys. Once a child is diagnosed with JM, it is always considered to be the juvenile form, even if the patient continues with the disease into adulthood
The immune system is a group of cells that normally protects the body from infection and other environmental factors. In an autoimmune disease like JM, however, the cells get active and they cannot stop. The immune system ends up attacking previously healthy tissues, harming the body instead of protecting it. In JM, the immune system mistakenly targets muscles, skin and other tissues.
The immune system attacks healthy tissue in JM this is called inflammation. In JDM patience, the inflammation focuses in the blood vessels that lie under the skin and in the muscles. With JPM, the attack is focused more against the muscle cells.
This inflammation causes the weak muscles and – in the case of JDM – skin rashes. If blood vessels or muscle cells in other parts of the body are inflamed, other systems of the body can be affected, such as the digestive tract, heart and lungs.

Although medications can help ease the symptoms of JM, the disease has NO CURE.
 The primary medications used to treat the symptoms of JM are corticosteroids, immunosuppressants and chemotherapy. These medications themselves can cause severe side effects, making JM challenging to treat. Many researchers believe that early and aggressive treatment is usually the best predictor of a better outcome of this disease.
High dose oral Prednisone or other corticosteroids often coupled with intravenous corticosteroids (Solumedrol) are usually the first line of treatment for JM. Since the side effects of corticosteroids can be very troublesome, Methotrexate (a chemotherapy drug administered at much lower doses than used for cancer patients) is usually introduced early to allow for tapering of the corticosteroids.
Second-line treatments include Cyclosporine and Intravenous Immunoglobulin (IVIG). Plaquenil is often used to control the rash.
For patients who do not respond to the first and second line of treatments, or in patients severely affected by the disease, Rituximab, Cytoxan and CellCept can be added. Protection from UVB is essential with sunscreens. Vitamin D with adequate calcium intake keeps bones strong.
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
In addition to medications, physical therapy is generally prescribed to increase strength and flexibility in muscles and joints.


There is no cure for JM, but with advances in early diagnosis and aggressive treatment, the outcome has continued to improve. Some children experience a mild form of the disease and may go into remission. Others follow a more severe and potentially debilitating course that can be life-long. Some JM patients will have a loss of range of motion. Some will battle an array of serious complications, resulting in the inability to walk, ongoing pain, disfigurement and even death. Whether the course of the disease is mild or severe, JM is life-changing for all of these children and their families.

INFO gathered from Curejm.org