Myositis can cause profound disability. What can be done?

It’s almost clinical, the way Bruce Wooderson lists the impact of his rare and progressive muscle disease.
 
Muscle deterioration in the arms, fingers, throat, abdomen and thighs, leading to significant disability. Unable to cut up, or even swallow, much of his own food. Unable to walk unaided, with many associated falls. Difficulty using the toilet. Regular hospital attendances. 
 
The NSW man’s disease, inclusion body myositis (IBM), is one of several forms of myositis.
 
Another type, polymyositis, typically presents with difficulty in muscles close to the trunk and affects daily tasks like hanging washing or drying hair and walking up steps.
 
Dermatomyositis can present with skin rashes, pain, severe lung issues and weak muscles close to the trunk.
 
Rarer forms of the disease such as necrotising myositis can come on very quickly and painfully, and the necrotic muscles may require surgery.
 
These diseases tend to come on later in life and occur when the body’s immune response attacks affected muscles and causes progressive degradation. There is currently no cure for any of the forms, though a few treatments exist for some forms of the disease.  
 
Yet despite its severe impact, myositis has a low public profile compared to the far better known motor neurone disease. Between nine and 50 people per million are thought to have the condition.
 
Given its rarity, myositis is often diagnosed late, according to Christine Lowe, President of the Myositis Association Australia.
 
‘The average time to diagnosis is around five years,’ she told newsGP.
 
‘Patients diagnosed with the treatable forms of dermatomyositis, polymyositis or necrotising are known to respond more quickly if treated early in the disease.
 
‘Those diagnosed with IBM, for which there is no current treatment, will cope better overall if they’re encouraged to undertake and maintain daily exercise from an early point.’
 
Ms Lowe said some of the association’s members were initially diagnosed with motor neurone disease and others as muscular dystrophy, while some have simply had it put down to ageing.
 
‘If people are having trouble getting off a chair, off the floor or the toilet, having falls or trouble raising their arms, alarm bells should ring. Those are red flags,’ she said.
 
Ms Lowe’s own diagnosis took six years. Her legs started feeling fatigued and she had trouble keeping her balance. Her GP put her through a battery of tests, but it wasn’t until she realised she could no longer keep up with other people while walking that a referral to a neurologist led to a muscle biopsy and her diagnosis of interstitial myositis.
 
Ms Lowe counts herself comparatively lucky, given steroid treatment is partly effective for her form of the disease and has slowed deterioration.
 
‘These diseases are linked to significant psychological issues,’ she said. ‘Patients are usually perfectly healthy and robust when myositis arrives, either in a creeping fashion or more dramatically.
 
‘They struggle to accept that their body is starting to let them down.’
 
For Mr Wooderson, myositis has taken more than his muscle strength. The 80-year-old’s greatest loss is music. He can no longer play the violin, organ and piano, or sing in choirs. His relationship with his wife has been affected. Many social activities are now off limits.
 
And all of this has taken a toll on his psyche.
 
‘Feelings of worthlessness and despondency do affect me emotionally,’ he said. ‘My wife’s understanding and patience is my greatest asset.’
 
Two trials about to start in Australia offer glimmers of hope for people with IBM. One is for the use of rapamycin in a phase 3 trial, and another phase 1 trial of a new compound. Drug trials are taking place overseas for other forms of the disease.
 
But while these trials give some room for optimism, any results will take years.
 
Ms Lowe understands the difficulty of early diagnosis, given many GPs will go their entire careers without seeing a myositis patient. She is keen to raise awareness of the condition.
 
‘Through our organisation, I see the wide presentation of the disease,’ she said.
 
‘For those with IBM, which has no treatment, life can become incredibly small. It’s like motor neurone disease in its progression, but it’s not fatal and patients typically live a long life.
 
‘We need support for our patients and their families along their journey.’
 
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muscular disease myositis