Madeline was diagnosed exactly one year to the day today. I can still remember that out of body experience when hearing her diagnosis from Dr. B. Looking back, it seemed our life would never be the same again. Not the same for our family, and especially Madeline.
In the beginning, we didn't know what we were dealing with. And most physicians I know didn't know much about the disease either. That implemented fear. Fear of the unknown - the worse of all fears. Our luck did change with finding a doctor not too far away. We trusted his judgement and so far his medication regiment has paid off.
The first six months of her treatment plan were tough. Besides the side effects of the medications, her neurological effects and personal issues at school, we (her mom and dad) were having a very tough time adjusting to this reality. We didn't know where this was going to take us. And the most concerning thought...where is this disease going to take Madeline. Some of the most horrible thoughts crossed my mind...I could not lose this baby, nor was I going to sit around and hope she gets better. We'd find the best, buy the best and do what's best for her. And we did.
Madeline is back to her old self now...a year from diagnosis. She no longer looks ill. She no longer has the grottron's papules or visual rashes. She looks like a normal, healthy and happy 10 year old girl. She dances and shines on stage like she once did. She doesn't tire as badly as she did six or even three months ago. Is she in remission? No. And I know that. But is she in a better place than she was this time last year? Hell YES! And we are too...as a family. The horrible aspects of this disease has done one thing for all of us - made us thankful of today and thankful for each other. I hope I am a better mother to Madeline and a better person to friends and family. And I know Madeline will be a stronger and wiser young lady one day because of her challenge w/ JDM.
I started this blog as therapy for me. Being my personality is somewhat OCD..I needed to manage this disease in some fashion. And this is how I could do it. But hopefully other shocked, scared, newly diagnosed childs' parents can find this blog online and realize it does get better. Your child can get better. Thank God for modern medicine and the wonderful medical professionals who research ongoing to find better answers for their care.
In just one year...we've come a long way. This one year mark reminds us of this. And we are thankful. I am thankful.
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Diary of daughter with Juvenile Dermatomyositis
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About Me
- Madeline's Juvenile Dermatomyositis
- This is a site where our daughter, Madeline, can recall her experiences with her very rare disease called Juvenile Dermatomyositis. I've decided to develop this blog to chronologically keep up with her progress for physician visits. Plus, as an outlet to journal our families challenges, fears and sucesses while dealing with her illness. Madeline is a wonderful, beautiful 11 year old girl who was diagnosed with JDM on March 28, 2008, when she was 9. She loves her teacup poodle Lulu, dancing, swimming, art and friends. She wants to be an LSU Golden Girl one day as well as a mommy.
Want to contact Madeline or her parents?
We too looked online to learn all we could about this rare disease. It is so hard to learn about any illness regarding your child, but when there are less than 3,000 children in the U.S. currently battling the disease the information and research is even more difficult to find.
You are welcome to email us at coachrickd@gmail.com
You are welcome to email us at coachrickd@gmail.com
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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.
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).
Prayers
And we can be confident that He will listen to us whenever we ask Him for anything in line with His will. And if we know He is listening when we make our requests, we can be sure that He will give us what we ask for.
1 John 5:14-15Please pray for Madeline and the rest of our family.
1 comment:
I thank you for this blog - I was one of the "shocked and scared" parents who found this blog - it really helped to read and understand that I wasn't alone in all of this! Best of luck to you and your beautiful girl with the "Julia Roberts" smile!!
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