Research

Patient vitals (especially body temperature), lab results, etc. may be out of reference range. Inquire about baseline, and trust patients/parents as experts on their own “normal” values.

Prevent whenever feasible, otherwise treat quickly and aggressively:

• Fever/Infection

• Dehydration

• Fasting

• Overheating

• Hypothermia

Many patients with LBSL are particularly vulnerable to severe consequences from head injury. Recommend thorough neurological assessment, extended observation, and low threshold for imaging.

 Diligently look for source of fever or symptoms suggestive of infection; treat aggressively. Recovery may be slow.

Anaesthesia may cause motor crises/delayed recovery. Patients may be taking custom prescription “mito cocktails” (high potency antioxidants and amino acids) to support metabolic needs. Consult with pharmacist and/or clinicians familiar with mitochondrial disorders and treatment. Additional labwork may be indicated.

Patient recovery may be longer than expected. Plan for extended impact from surgery, anesthesia, illness, injury, aggravating conditions (see above), and/or change in medication.

Patients should be counseled to follow up with their primary care provider, neurologist, and/or metabolic specialist soon after discharge. Follow-up lab work may be indicated. Consult OT/PT as needed. Refer patient to new specialists as needed to complete care team.