Caleb, my eldest child had me convinced I had hurt him mortally when he was diagnosed with JDM.
At the end of spring in 2008 my 6-year-old son started to refuse walking up the stairs to our house. He insisted I carry him because his knees hurt too much. I humoured him thinking it was a regression and attention seeking because his little sister being born in June.
Caleb was tall for 6 so I automatically guessed it was growing pains. In the back of my head I thought growing pains at 6 was a little early. He then began losing weight. Not much at first. But being my first born I was overly vigilant and noticed. This all happened over a period of about 2 months.
Something is wrong
Early December I had asked the GP for a referral to a Paediatrician at the local hospital. School ended before we saw the Paediatrician. His teacher informed me of Caleb’s inability to take off his wet clothes after swimming lessons unassisted. This information was important, and she waits until the end of the year to tell me. It was completely out of character for my boy.
When we got to see the paediatrician Caleb was worse. He was tired most of the time furthermore he couldn’t walk for very long and struggled to do normal everyday things.
A good Doctor comes in handy
I think we got lucky with the paediatrician owing to a fast diagnosis. I described the symptoms to him then he had a quick look at Caleb; the doctor gave me a preliminary diagnosis of Juvenile Dermatomyositis, JDM. Now as they told me make sure you put Juvenile in first when you look it up. The doctor ordered an MRI to confirm his diagnosis. If that was inconclusive Caleb would have to undergo a skin and muscle biopsy. They performed the MRI that day. Because of Christmas we had to wait a full 2 weeks to get the results.
What is JDM
JDM is an autoimmune inflammatory disease of the muscle (myositis), skin, and blood vessels. Symptoms range from mild muscle weakness such as difficulty rolling over in bed to severe muscle weakness enjoy having trouble swallowing. There is a rash that goes with it. The skin rash can be a mild redness to an ulceration formation.
All groups being affected by JDM equally. The disease usually presents during childhood. Roughly between the ages 5 -10. Girls being affected about twice as often as boys. JDM is rare with approx. 2-4 children per one million children being diagnosed each year in the United States.
In most cases the cause of JDM is unclear. For some unknown the immune system turns on the muscles and damages muscle tissue. In JDM the cells attack the small blood vessels supplying the muscles and skin.
The Results and Diagnoses
In early January 2009 our appointment came. Caleb had lost further strength and could barely get out of a seated position. The doctor’s diagnosis confirmation of JDM came through as they gave us a choice of hospitals. Brisbane Women and Children’s Hospital or Westmead Children’s Hospital for treatment.
My head was reeling. My healthy, bright boy was fast becoming incapacitated because of me. I blamed myself and the horrible feeling that I caused this grew and persisted for a long time.
There was no choice for us. I chose Westmead as it was close to my parents and family. At the time I desperately needed family around. We packed our house up and left for Penrith within the week. They scheduled our first stay in the hospital for the beginning of February.
Westmead Children’s hospital for JDM treatments
They allocated Caleb a bed in a ward and his treatment began immediately. The very first appointment was with the physio department.
I knew Caleb was weak but during that initial physio session I learnt just how weak. He scored a 3 out of 52 on a specifically designed strength assessment. The assessment was simple. It comprised basic things, such as lifting your head off the floor, or getting up without using aids. Basically, all he could do was walk. It was a very scary moment.
After the physio appointment Caleb’s scheduled drip feed rounds of medication began. The medication was massive doses of corticosteriods. It knocked Caleb around significantly. He was barely conscious for the next 9 days during the three large doses.
Using Corticosteroids in treatment of JDM
Treatment for JDM aims to minimize inflammation, improve function, and prevent disability. The treatment should be early. JDM requires a team approach between the Rheumatologist, physical therapist, dermatologist, and primary care doctor. JDM is unique because doctors can cure it.
Corticosteroids alter the immune system by limiting the production of antibodies and reducing inflammation therefore improving muscle strength. The steroids, especially prednisone, are usually the first choice because they work quickly.
The Application of JDM medications
A very high dose to start treatment, often intravenously. We will see improvements in about two to four weeks as inflammation diminishes. Standard treatment for JDM is high-dose daily oral prednisone in divided doses. Continued improvements should be clear. They reduce the dose over a two-year period. They will discontinue Prednisone before the two-year period.
Patients will develop treatment-related side effects. The side effects are wide ranging, like weight gain, osteoporosis, and cataracts. Doctors recommend supplements like calcium and vitamin D to strengthen bone and regular eye exams to detect cataracts.
Other medications work slower and have fewer side effects. The other medications enable patients to wean off steroids sooner. Methotrexate is the best initial treatment for most JDM subjects and is now started with the corticosteroids.
Sun protection goes hand in hand with treatment. UVA and UVB light adversely affects the skin rash. The best option is to avoid the sun during peak daylight hours and slip slop slap.
Physio appointments are essential to maintain and to improve strength. As well as to prevent muscle wastage and calcium deposits developing.
Speech therapy and diet assessment are important sometimes with JDM cases.
I will continue with part 2 another time. There is far too much to go to try and cram it all in now.