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Intrathecal baclofen Best practices project Troubleshooting Michael Saulino, MD PhD Physiatrist MossRehab Elkins Park My Disclosures • Speaker’s bureau and clinical investigator for Jazz Pharmaceuticals • Speaker’s bureau and clinical investigator for Medtronic, Inc • Clinical investigator for Mallinckrodt • Consultant for SPR therapeutics • NANS Board of Directors Objectives / Outline • Review the techniques for evaluation and management of optimal intrathecal baclofen therapy • Under-dosing • Over-dosing Important Caveat • While this presentation (and related manuscript) focus on device related issues, it is important to recognize non-device related issues that can present in a similar fashion • Noxious stimuli can drive increased tone • Disease progression or exacerbation can mimic over or under dosing Diagnostic evaluation process • Targeted medical history – onset, course, duration, exacerbating / relieving factors of current presentation, associated symptoms – Recent intrathecal history – last refill, last dosing adjustment, last surgery, etc. • Focused physical examination – Vitals – Neuromuscular exam – Item relevant for potential noxious stimuli Diagnostic evaluation process • Judicious use of radiologic/laboratory testing – Noxious stimuli search – CK levels – Newer Medtronic catheter is radiolucent • Obtain current telemetry – Compare to prior history – Consider a bolus as a combined diagnostic / therapeutic maneuver • Check reservoir volume and compared to predicted volume Diagnostic evaluation process • Catheter access port (CAP) aspiration • Catheter dye study vs. CT myelogram with dye injection through CAP • Nuclear medicine cisternogram • MRI of thoracic spine Differential diagnosis: under dosing • • • • • • • • Other medications Sepsis Meningitis Neuroleptic malignant syndrome Autonomic dysreflexia Serotonin syndrome Malignant hyperthermia Seizure disorder Differential diagnosis: overdose • • • • • Other medications Sepsis Intracranial hemorrhage Hypoglycemia Increased intracranial pressure (Cushing’s triad) • Electrolyte imbalance • Seizure disorder Under dose/withdrawal management • Treatment occurs prior to or in parallel with diagnostic workup • Patient require monitoring (local resources) • Tossup – triage/stabilize remotely vs. immediate transport • First line therapy: restoration of intrathecal baclofen delivery – Single vs multiple boluses – LP, external catheter, new implanted system Under dose/withdrawal management • Restart dosing based on intensity and duration of symptoms • It intrathecal delivery cannot be achieved promptly, oral medication can be used as temporizing measure • Oral baclofen is agent of choice but should not be relied on to halt progressive withdrawal • No uniform oral to intrathecal conversion • Additional options include benzodiazepines (IV) and cyproheptadine Overdose management • • • • • • Support airway, respiration and circulation Reduce or stop ITB delivery Option: CSF drainage Advise against physiostigmine Watch for rebound withdrawal Determine underlying cause Emergency preparedness • Structured, consistent on-call system including off hours coverage • Direct communication between the managing clinician, implanting surgeon, emergency department, and critical-care team • Patients should be educated on the signs and symptoms of over- and under dosing • Patients in distress should call their managing physician AND go directly to the ED Summary • Patients demonstrating suboptimal effects of ITB therapy should be evaluated promptly with a focused medical history, targeted physical examination, system interrogation, and radiologic testing. • Patients can demonstrate adverse effects from both over- and underdo sing. Clinicians managing these patients should be familiar with the evaluation and management of these syndromes. Summary • Providers of ITB therapy should create on-call and triage protocols for management of adverse effects. Patients should be educated on how to engage these protocols.