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Steroid dosing in Duchenne can be a confusing topic – what is enough, what is too much, why weekend doses are higher. With these frequently asked questions I’ve heard from the community in mind, I reached out for input from Dr. Doug Biggar and Dr. Anne Connolly, who contributed to the information below. So, with all of this amazing expertise, let’s sift through this together.

* NOTE: Remember to ALWAYS consult your physician before making any changes to your current dosing regimen.

How are steroids used in Duchenne? 


Synthetic glucocorticoid medications (“steroids”) are standard of care for people living with Duchenne, and include prednisolone, prednisone, and deflazacort (Calcort, Emflaza). Steroids have been prescribed and taken in many doses and using many different schedules, but in the US, dosing is either daily or high dose weekends; in other countries, other dosing schedules (i.e, 10 days on, 10 days off, etc.). The impact that steroids have on the body depends on the dose of the steroid and how often the steroids are taken.

 

For some people, daily steroids may result in excessive weight gain, behavior issues, osteoporosis, cataracts, slowing of growth, pubertal delay, and gastric upset/ulcers. People who are taking intermittent doses (i.e., not daily) appear to have fewer of these problems. 

What are some key steroid management facts to remember? 

 

All people taking steroids are encouraged to:

  • Take steroid medication with food
  • Use anti-acids/proton pump inhibitors as needed to manage stomach upset
  • Check weight and height/lengths at every visit
  • Check eyes yearly for cataracts
  • Have a 25 OH vitamin D level drawn at least yearly
  • Review diet with a nutritionist to make sure diet is appropriate for calories and is high in calcium
  • Check bones (DEXA scan every 2-3 years and spinal x-ray every 1-2 years) to monitor for osteoporosis
  • Manage behaviors as needed
  • If you take daily steroids, do not miss steroids for more than 24 hours
  • Especially if you take daily steroids, be sure to receive stress doses as needed. If there is ANY thought that a stress dose might be needed, it is better to take the stress dose than to not take it. All information can be found on the PJ Nicholoff Steroid Protocol

 

Is there a maximal dosing of steroids in Duchenne?

Prednisone is dosed at .75mg/kg/day; deflazacort is dosed at .9mg/kg/day. Due to the effects of daily steroids on the body, as well as the risk of side effects with high doses, daily doses of steroids are generally not prescribed in doses higher than around 36mg/day of prednisone (or 40 mg/day of deflazacort). While the FOR-DMD study is looking at dosing, there currently is no information around maximal dosing of steroids in Duchenne. Generally doses of twice weekly steroids can go up to 250mg/day of prednisone (or 300 mg/day of deflazacort, which is comparable to the prednisone weekend dose, but has not been studied) given on weekends. 

 

If you receive a prescription for steroid doses that are higher than 36mg/day of prednisone, 40 mg/day of deflazacort, 250mg/day for twice weekly prednisone, or 300mg/day for twice weekly deflazacort, or if you have ANY doubt, please call your neuromuscular specialist to make sure your dose is correct.

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