The recommended starting dose of prednisone is 0.75 mg/kg/day and that of deflazacort is 0.9 mg/kg/day, given in the morning. Some children experience short-lived behavioural side effects (hyperactivity, mood swings) for a few hours after the medication is given. For these children, administration of the medication in the afternoon may alleviate some of these difficulties.
For ambulatory individuals, the dosage is commonly increased as the child grows until he reaches approximately 40 kg in weight. The maximum dose of prednisone is usually capped at approximately 30 mg/day, and that of deflazacort at 36 mg/day.
Non-ambulatory teenagers maintained on longterm steroid therapy are usually above 40 kg in weight and the prednisone dosage per kg is often allowed to drift down to the 0.3 to 0.6 mg/kg/day range. While this dosage is less than the approximate 30 mg cap, it demonstrates substantial benefit.
Deciding on the maintenance dose of steroids is a balance between growth, how good the response to steroids is and the burden of side effects. So this decision needs to be reviewed at every clinic visit based on the result of the tests done and whether or not side effects are a problem that can’t be managed or tolerated.
In boys on a relatively low dosage of steroids (less than the starting dose per kg body weight) who start to show functional decline, it is necessary to consider a “functional rescue” adjustment. The dosage of steroids is increased to the target and the individual is then reevaluated for any benefit in approximately two to three months.
There is no consensus on the optimal steroid dosage if initiated in the non-ambulatory individual. Nor is it known how effective steroid treatment is in preventing scoliosis or in stabilising cardiac or respiratory function in this setting. This issue warrants further study.
Information based on consensus statement (published in January 2010)