Standard of Care Summaries

Recommendations based on the PJ Nicholoff Steroid Protocol

Timeline and dosing

Initial discussion

Discuss use of steroids with family
Begin steroid regimen
- Before substantial physical decline
- After discussion of side-effects
- After nutrition consultation
Recommended starting dose
- Prednisone or prednisolone 0.75 mg/kg per day
OR
- Deflazacort 0.9 mg/kg per day
Dosing changes

If side-effects unmanageable or intolerable
- Reduce steriods by 25–33%
- Reassess in 1 month
If functional decline
- Increase steroids to target dose per weight on the basis of starting dose
- Reassess in 2–3 months
Use in non-ambulatory stage
- Continue steroid use but reduce dose as necessary to manage side-effects
- Older steroid-naive patients might benefit from initiation of a steroid regimen

Cautions

Adrenal insufficiency

Patient and family education
- Educate on signs, symptoms, and management of adrenal crisis
Prescribe intramuscular hydrocortisone for administration at home
- 50 mg for children aged <2 years old
- 100 mg for children aged ≥2years old and adults
Stress dosing for patients taking >12 mg/m² per day of prednisone / deflazacort daily
- Might be required in the case of severe illness, major trauma, or surgery
- Administer hydrocortisone at 50–100 mg/m² per day
Do not stop steroids abruptly
- Implement PJ Nicholoff steroid-tapering protocol
- Decrease dose by 20–25% every 2 weeks
- Once physiological dose is achieved (3 mg/m² per day of prednisone or deflazacort) switch to hydrocortisone 12 mg/m² per day divided into three equal doses
- Continue to wean dose by 20–25% every week until dose of 25 mg hydrocortisone every other day is achieved
- After 2 weeks of dosing every other day, discontinue hydrocortisone
- Periodically check morning CRH-stimulated or ACTH-stimulated cortisol concentration until HPA axis is normal
- Continue stress dosage until HPA axis has recovered (might take 12 months or longer)