INSTRUCTOR GUIDE FOR BACLOFEN WITHDRAWAL SIMULATION SCENARIO

I. Title: Baclofen pump failure/Baclofen withdrawal

II. Target Audience: Healthcare providers and teams who care for pediatric health emergencies, including but not limited to pediatric and emergency medicine residents, pediatric emergency medicine fellows, pediatric and emergency medicine physicians, nursing, respiratory therapy, and other allied health professionals.

III. OverviewThis case centers on an adolescent patient with altered mental status, agitation, and suspected intrathecal baclofen pump failure. Learners should recognize and appropriately manage suspected baclofen withdrawal, manage agitation and spasticity, demonstrate appropriate airway and circulatory management, appropriately consult subspecialists (including Physical Medicine and Rehabilitation and/or Neurosurgery), and provide patient disposition to a pediatric critical care unit.

IV. Purpose

This case was designed to teach emergency medicine physicians, pediatricians, and other healthcare providers about the clinical presentation, stabilization, and medical management of the patient with spasticity and agitation associated with suspected or proven baclofen withdrawal. Simulation was chosen because, in contrast to traditional lectures, it allows for team-based practice and fine-tuning of a systematic approach to acutely ill patients in a safe learning environment. The overall goal is enhanced recognition and management of pediatric patients with high-acuity, low-frequency toxicologic emergencies.

V. Conceptual background

Prior to development of this simulation scenario, our fellowship included lecture-based teaching on toxicology topics based on the ACGME PEM core content outline. This case was developed to ensure a systematic opportunity for each trainee to practice acute recognition, differential diagnosis, evaluation, and management of toxicology emergencies.

A group of content experts from PEM and toxicology reviewed ACGME content guidelines for toxicology. This scenario was written initially by a single author, reviewed by multiple authors for content and clarity, piloted prior to the actual simulation day, and revised based on feedback.

VI. ImplementationThis case requires use of Laerdal SimMan 3G and its associated software and program files, however a low-fidelity mannequin and simulation environment could also be used if verbal

cues could be provided by instructors throughout the scenario. This scenario was conducted in a resuscitation room equipped with standard pediatric resuscitation equipment, including airway equipment, monitors and defibrillators, IV access, and standard resuscitation drugs. Participants were oriented to the mannequin and the resuscitation equipment prior to the scenario. This scenario was designed to take 10-15 minutes to complete with 30 minutes immediately following to debrief.

Scenario participants include 2-3 physicians and a nurse (who is also one of two confederates). One facilitator is needed to run the Laerdal software and 1-2 facilitators should serve as over-the-phone consultants and conduct a debriefing with the learning materials provided. This scenario calls for two confederates: one to play the role of the resident physician providing patient handoff and one nurse. The nurse confederate should have direct communication with a facilitator (via earpiece or in person) to assist in prompting participants when/if deviation from the scenario algorithm occurs. The nurse confederate is given notecards printed with specific physical exam findings and lab values to hand to participants when prompted (see Appendix C). For our scenario, a cord attached from the mannequin’s arm to a bicycle brake was used to simulate spasticity, however this may also be provided to participants using notecards or verbal cues instead.

VII. LimitationsMental status, capillary refill, and the presence or absence of neurologic manifestations are often difficult to simulate. Notecards and verbal cues should be provided by the confederate for clarity. Our workshop included only PEM fellows and nurses, and therefore has not been evaluated with other learner groups. We also did not include a didactic session with the scenario, but feel this may have been helpful to the participants’ overall learning experience.

VIII. Learning Objectivesa. Primary:

i. Recognition and management of child with severe agitation and altered mental status

ii. Recognition and management of intrathecal baclofen (ITB) pump failure and withdrawal

iii. Reevaluation and care escalation of the decompensating patient with a labeled diagnosis at sign-out

iv. Assignment of team roles, including team leader b. Secondary:

i. Demonstrate appropriate airway managementii. Demonstrate appropriate circulatory support

iii. Demonstrate closed-loop communication and shared mental model

iv. Demonstrate appropriate subspecialty consultation with Neurosurgery, PM&R, and Toxicology/Poison Specialist

v. Provide appropriate patient disposition and transition of carec. Critical Actions Checklist (see Appendix B, checklist)

i. Establishment of team roles, including team leaderii. Sign-out is received clearly by oncoming team with verbal review of lab work

and radiologic studies performediii. Receptive and responsive to nursing staff’s concerns for patient’s increasing

agitationiv. Recognition of persistent agitation and altered mentationv. Timely placement of cardiac monitors and vital signs

vi. Basic airway management (Oxygen administration, BVM)vii. Basic circulatory management (IV fluids administered)

viii. Considers differential diagnosis of altered mental status, agitation, and fever, including ITB failure, baclofen withdrawal, and infection

ix. Treatment of agitation and withdrawal symptoms with benzodiazepines and/or baclofen

x. Additional labs requested (CK, u/a, urine myoglobin, blood culture)xi. Recognition and appropriate management of rhabdomyolysis with IV fluids and

sodium bicarbonate administration, antipyreticsxii. Poison Center or Toxicology consultation

xiii. NSGY and PM&R consultationsxiv. Disposition to pediatric intensive care unit

d. Optimal sequence of critical actions: expected sequence as abovee. Duration to critical actions: total scenario to be completed within 15 minutes of start

IX. Environment:a. Lab Set Up: This scenario has been run in the simulation lab in a large simulation room

with control room. It could also be run in an emergency room, including in a resuscitation or trauma bay, a decontamination area, or a regular room.

b. Mannequin Set Up: i. SimMan 3G (Laerdal) or similar adult-sized high-fidelity mannequin, with ability

to simulate seizureii. Female adult patient, exam gown, adult diaper with dark cola spilled inside (to

simulate hematuria), bilateral 6 mm pupilsiii. Lines needed: peripheral IV in place before scenario startiv. Baclofen pump: shallow circular lid or tin (approximately 4-5 cm diameter)

placed under manikin skin at region of right lower quadrant of abdomenv. Gastrostomy button (Mickey) trimmed and taped to left upper quadrant of

abdomen, 4x4” gauze trimmed and secured around gastrostomy buttonc. Props:

i. Basic airway and code cartii. Notecards with exam updates and lab results (see Appendix C)

iii. Xray: abdominal film with baclofen pump presentd. Distracters: Team must recognize that patient with the labeled diagnosis of “possible

appendicitis” (premature closure) at sign out requires reevaluation and escalation of care, and formulate a differential diagnosis for patient’s persistent agitation, fever, and tachycardia.

X. Actorsa. Roles:

Resident: provides sign-out to the team during rounds with script provided; portrays to the team that the patient likely has a diagnosis of appendicitis and does not mention baclofen pump failure as an alternate diagnosis.Nurse: informs team about patient progression after sign-out; keeps scenario flowing by handing notecards to team lead with lab updates, physical exam specifics, and pointing out dark urine in diaper (if not noticed by team by Act 4)

b. Who may play them: any medical provider with general experience in pediatric or emergency medical conditions

XI. Case Narrative (what the learner will experience)a. 17 year old female with cerebral palsy, developmental delay, seizure disorder, spasticity,

and intrathecal baclofen pump is brought to the emergency center by her parents for agitation, spastic episodes, and fever. Parents associate agitation with abdominal cramping/pain. Pt has been in the EC for 12 hours, during which the present team gave an initial IV fluid bolus and morphine (10 hours ago). CBC showed leukocytosis with neutrophil predominance, normal chemistries, stable CXR and KUB, and a right lower quadrant abdominal ultrasound that could not visualize the appendix. The patient has been awaiting abdominal CT. The current EC team is at the end of their busy shift and the resident provides sign-out to the oncoming team. The resident is frazzled and clearly overwhelmed by his busy shift, provides a very limited sign-out without mentioning differential diagnosis, and has not been back in to reassess the patient in 4 hours. Parents have left the EC to eat dinner and run errands; they told the nurse they would be back in about an hour.

After sign-out is complete, the EC nurse reports to the team that the patient is becoming more agitated with tachycardia and fever. After initial exam and basic interventions (oxygen, IV fluid bolus, antipyretics), team considers differential diagnosis of fever, agitation, tachycardia (including status epilepticus, encephalitis, infection, baclofen withdrawal). Dark urine in diaper, along with additional labs (u/a, CK) reveal signs of rhabdomyolysis. Treatments include IV fluids with sodium bicarbonate, IV benzodiazepines, and/or dantrolene or cyproheptadine. Xray abdomen reveals intact intrathecal baclofen reservoir and tubing. Physical medicine and rehabilitation team evaluates baclofen reservoir which appears to be full. Neurosurgery is consulted and will take patient to the OR for exploration. Poison Center and/or toxicology consultation will have no other suggestions. Pt will require pediatric intensive care unit admission.

b. Scenario Background given to participantsi. Chief complaint, triage note: painful episodes and “acting strange” for 2 days,

fever x 1 day ii. Past medical history:

Spasticity and seizure disorder secondary to traumatic brain injury from a motor vehicle accident at age 7. Developmental delay. History of constipation, asthma, and recurrent pneumonia. Pt followed by Neurology, neurosurgery, and physical medicine and rehabilitation.

iii. Past Surgical History: Gastrostomy button and fundoplication done at 7 years of age; per parents, the patient still has her appendix. Intrathecal baclofen pump placed by neurosurgery at 14 years of age.

iv. Meds and allergies: Levetiracetam 1000 mg per GT BID Polyethylene Glycol 17 grams per GT BID Ativan 1 mg per GT BID, prn agitation/spasticity Clonazepam 1 mg per GT QHS, PRN sleepAlbuterol MDI 6 puffs every 4-6 hours, as needed for wheezing/coughNKDA

v. Family/social history; lives with mom, dad, 11 y/o sister. Has a home health nurse daily on weekdays; the patient has been on vacation with her parents for the past 3 weeks.

c. Scenario conditions initially Act 1:Resident narrative: (at evening sign-out rounds…nervous, tired, and clearly overwhelmed after a busy shift): “This is a 17 year old female with a history of cerebral palsy from a traumatic brain injury at age 7 after an MVC presenting with agitation, fever, and rapid heart rate x 24 hours, seems to have abdominal pain associated with these episodes. PMH: CP, seizure disorder, recurrent pneumonia, last admitted for pneumonia 4 months ago. Also has a history of constipation, on daily bowel regimen; last stool was yesterday and was large and soft. She has a gastrostomy tube and fundoplication, and has a baclofen pump. She’s been in the ER for 12 hours, and so far she’s had a CXR that is negative for pneumonia, a KUB with a moderate amount of stool present, and a CBC with a WBC of 19K with left shift, and normal chemistries. Our concern is that she may have acute appendicitis, so we got an ultrasound, which was not able to visualize the appendix, so we’re waiting for CT.”(NO mention that this could be baclofen pump malfunction…fellow must sort through this information and consider other differential diagnoses for agitation and fever).

If prompted by team leader, resident actor should use the following scripted answers:

“Pain medication?”—she had a dose of morphine this morning. I haven’t seen her in a few hours, though. I’ll go back in and see her before I leave to see if she needs any more.“Antibiotics?”—Not yet. Do you want me to order some?“IV fluids?”—yes, she had a bolus when she first got here. Her heart rate was in the 170’s, but she had a fever with it. I think she’s on maintenance now.“Did they comment on the baclofen pump on the x-ray?”—I think so…oh, yeah. Radiology read it as stable with normal tubing. “Has PM&R been consulted?”—I paged them a couple of times, but I haven’t heard back“Has Neurosurgery been consulted?”—No. Um, I think we were waiting for the CT to come back first.“When was her last baclofen dose change?”—Her dose has been the same for a long time, I think. I’m not sure what her dose is.

d. Scenario branch points (see Appendix A, scenario algorithm)ACT 2 Nurse narrative: (rushes over to team after sign-out, clearly worried and frantic): “I need a medical evaluation in room 7! The patient is back from CT and is extremely agitated and having spastic episodes. She hasn’t been reassessed by a doc in almost 4 hours. I haven’t put her back on monitors yet since she just came back from CT, but she’s just so agitated and won’t stop crying! Her parents stepped out to get something to eat and I can’t get a hold of them!”

Initial Exam:Primary:

Vital Signs: HR 170 RR 30 T102 BP 130/90 Pox 95% RAAirway: patent, delayed gag reflex, thick oral secretionsBreathing: tachypneic with shallow respirationsCirculation: warm, clammy extremities, brisk capillary refill;

Secondary: HEENT: pupils 5-6 mm and reactive bilaterally, mucus membranes slightly tacky; poor oral hygieneLungs: clear bilaterally; tachypneic with shallow respirationsCardiac: tachycardic, no murmur or gallopAbdomen: abdomen slightly tense during spasms, gastrostomy site clean and dry without obvious signs of infection; baclofen pump site clean and dryExtremities: spasticity noted in upper and lower extremities; Skin: warm, diaphoretic; brisk capillary refillNeurologic: Pt is alert but extremely agitated, cries intermittently, hyperactive startle reflex

ACT 3: Pt has a 1-minute spastic episode (seizure mechanism); she is crying and moaning in pain. Monitors should be placed. Pt should be given oxygen, IV fluids, and benzodiazepines. Differential diagnosis list should be verbalized.

ACT 4: Nurse actor informs team that physical medicine and rehabilitation has assessed the baclofen pump and the reservoir is full, and they suggest neurosurgery evaluation (if not already done by then). If not recognized by participants, nurse actor points out that there is dark brown urine in diaper.

Labs on notecard provided to team lead by nurse actor when requested. Treatments include NS bolus, IV fluids with bicarbonate, benzodiazepines, antipyretics, baclofen via gastrostomy tube.

ACT 5: Pt calms with benzodiazepine and baclofen therapy; Toxicology and/or Poison center consultation; ICU consultation and transfer

XII. Instructors Notes (what the instructor must do to create the experience)a. Tips to keep scenario flowing in lab and via computer

See triggers located on Scenario Algorithm, Appendix A, for changes in vital signs and mannequin findings.Nurse actor provided with a headset to receive information directly from those running the computers/manikins. Actors are also given a set of notecards (Appendix C) to hold during scenarios with lab results and patient descriptors not apparent to the learners (e.g. capillary refill < 3 seconds; patient is agitated). Actors are directed over headset to give participants specific cards if prompted

b. Tips to direct actors- Nurse actor provided with a headset to receive information directly from those running the computers/manikins. Actors are also given a set of notecards (Appendix C) to hold during scenarios with lab results and patient descriptors not apparent to the learners (e.g. capillary refill < 3 seconds; patient is agitated). Actors are directed over headset to give participants specific cards if prompted.

c. Scenario programming-i. This scenario is easily run “on-the-fly” (without a program) by an experienced

mannequin operator. The scenario management path, as well as potential complications and errors, are shown in Appendix A.

XIII. Debriefing Plana. Method of debriefing-The scenario was debriefed by physician simulation instructors

who had been trained in debriefing methods including debriefing with good judgment and Advocacy-Inquiry. In addition, pediatric toxicologists participated in the debriefings as content experts.

b. Actual debriefing materials- See Appendix B for Critical Actions Checklist, Appendix D for Debriefing Content.Instructors watched the scenario using the Critical Actions Checklist, and used that to guide their debriefing.The Debriefing Content form summarized the key medical information about baclofen overdose and withdrawal.

c. Rules for the debriefingi. Basic Assumption-All learners are motivated, intelligent adults who are already

well-trained, and are participating in the simulation to further their learning. Scenarios are chosen because they provide challenges that may be uncommon

clinically, difficult, or critical. Mistakes are expected and welcomed as learning opportunities.

ii. Confidentiality-Performance in all simulations is confidential. The specific contents of the simulation should also be kept confidential.

iii. Fiction Contract-All learners and instructors agree to suspend disbelief and work to make the scenario as realistic as possible, recognizing that simulation is not the same as reality.

iv. Debriefings took place in a separate room from the scenario, and had video available for review as needed.

XIV. Pilot Testing and Revisionsa. Pilot Testing-completed at the Texas Children’s Hospital Simulation Center on July 21st,

2012.b. Participants: A total of 12 pediatric emergency medicine fellows and 4 pediatric

emergency medicine nurses participated in the scenario, divided into 4 groups. At least 2 simulation faculty and 2 personnel members per scenario are required for a successful simulation experience.

c. Performance expectations, anticipated management mistakes-Participants were informed that this was a non-graded interactive learning experience (similar to other simulation days they had previously experienced). They were informed that scenarios were chosen specifically to be challenging and that mistakes are both expected and welcomed as learning opportunities.

d. Evaluation form for participants-All participant filled out a course evaluation at the end of the simulation day, which included specific feedback about scenarios, content, and instructors. Based on this feedback, scenarios were modified for future use.

e. Scenario Revision: scenario revisions were performed in May 2013 and May 2014XV. Authors and their affiliations

a. Elaine Fielder MD, Department of Pediatrics, Section of Emergency Medicine, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX.

b. Daniel Lemke MD, Department of Pediatrics, Section of Emergency Medicine, Baylor College of Medicine, Texas Children’s Hospital, Houston, TX.

c. Cara Doughty MD, MEd, Department of Pediatrics, Section of Emergency Medicine Baylor College of Medicine, Texas Children’s Hospital, Houston, TX.

nScenario Timeline Vital Signs/PE Facilitator Information

Act 2: Nurse, nurse!

Timer: 3-5 minutesTriggers: 3 min into scenarioEnd: 5 min into scenario

Act 3: Agitation increases

Timer: 5-8 minTriggers: 5 min into scenario End: 8 min into scenario (or when benzodiazepines administered)

Act 4: Coca-Cola urine

Timer: 8-13 minTriggers: 8 min into scenario or after benzodiazepines givenEnd: 13 min (or when IV fluids/bicarbonate administered for suspected myglobinuria)

Act 5: Improvement and transfer

Timer: 8-13 minTriggers: benzodiazepines givenEnd: 13 min into scenario or when PM&R, neurosurgery, and toxicology consultations complete

Act 1: Sign-Out

Start Timer: 0-3 minHistory provided by resident at evening sign-out

VS: HR 170 (sinus) RR 30 T102 BP 130/90 95% RA

Agitated, spastic movements intermittently with periods of crying, acts like she is in pain.

VS: HR 180 (sinus) RR 30 T103 BP 130/90 93% RA

Continued agitation, 1-minute spastic episode (mannequin seizure); crying in pain; hyperpyrexia.

HR 180 (sinus) RR 30 T103

BP 130/90 93% RA (96% facemask)

Pt with continued agitation, crying, hyperpyrexia; tachycardia continues

Dark urine in diaper noted by nurse

HR 120 RR 22 BP 120/70 96% on facemaskImproved agitation

Sign-out by resident with presumed appendicitis: u/s inconclusive, waiting for CT results

CBC: WBC 19K, 65%neutrophils, 10%bands

Additional information given if requested

EC nurse calls for medical evaluation because of increased agitation and spasticity

Team to perform initial exam on patient at this time

Monitors, pulse ox, vital signs Additional labs requested

Verbalize differential diagnoses and etiologies of agitation

Benzodiazepine and/or baclofen administration

Antipyretics, oxygen, IV fluids PM&R/NSGY consults

Dark urine pointed out by nurse if not recognized by participants

Labs (notecards):o Chemistry: Na 140 CO2 16, Glucose

135; all other normalo CK: 100,500o u/a: 1.020, pH 6.4,

-nitrites/leukocytes, 3+blood, 1+protein; RBC: 0-4; WBC: 0-

o urine myoglobin 3+ IV fluid administration with bicarbonate

PM&R confirmed baclofen reservoir full Neurosurgery consulted for OR exploration Critical Care consultation Poison Center and/or toxicology consulted ICU transfer

Pt not on monitors at sign-out, patient recently returned from CT and is moaning in pain.

Appendix A: Scenario Algorithm

Appendix B: Critical Actions Checklist

Step Time Completed

Comments (Who, Praise, Problems, Gaps)

Establishment of team roles, including team leader

Clear sign-out obtained by oncoming team, including verbal review of labs and radiologic studiesResponsive to concerns of nursing staff regarding patient's worsening agitationRecognition of persistent agitation and altered mentationCardiac monitors placed, vital signs obtained

Basic airway management (O2 administration)

Basic circulatory management with IVF bolusConsiders differential diagnoses of agitation, fever, and tachycardia, including baclofen withdrawal Management of agitation/spasticity with benzodiazepine and/or baclofen administrationAdditional labs requested (CK, u/a, blood culture)Appropriate management of rhabdomyolysis (IV fluids with sodium bicarbonate)Appropriate consultation with PM&R and Neurosurgical teamsToxicology/Poison Center consultationPt transferred to pediatric ICU

Appendix C: Notecards with exam updates/lab results1. Mental status: agitated, crying2. Capillary refill: brisk 3. Labs:

a. Chemistries: Na 140 CO2 16, Glucose 135; all others normalb. CK: 100,500c. CBC: WBC 19K, 73 segs, 6 bands, 15 lymphsd. Liver panel: normale. Urine analysis: 1.020, pH 6.4, -nitrites/leukocytes, 3+blood, 1+protein; RBC: 0-4; WBC:

0-4, urine myoglobin 3+4. Radiologic studies:

a. CXR-normalb. KUB: moderate amount of stool in colon, no air fluid levels; baclofen reservoir in placec. Abdominal ultrasound: unable to visualize appendix

Appendix D: Debriefing Content

a. Baclofen mechanism of action: Synthetic derivative of GABA (g-aminobutyricacid). At therapeutic doses, it acts

principally on the GABA B receptors at the spinal/thalamic level, reducing the postsynaptic potentials along α motor neurons and thus relaxing the muscles

Commonly used in patients with spasticity from neurologic injury, cerebral palsy, etc.b. Oral baclofen

Does not effectively cross blood–brain barrier (lipophobic) Some patients need higher oral therapeutic doses to relieve spasticity High doses are related to systemic side effects (dizziness, weakness, tiredness,

headache, trouble sleeping, nausea, constipation) Patients who fail to respond to oral baclofen or who have significant side effects may

benefit from Intrathecal Baclofen (ITB) c. Intrathecal Baclofen (ITB)

Basic overview of baclofen pump:o Programmable implanted drug infusion system consisting of a pump and a

catheter Pump: round metal disc with a reservoir that stores and releases

prescribed amounts of baclofen through the catheter; measures approximately 1 inch thick x 3 inches in diameter; surgically secured under the skin of the lower anterior abdomen

Catheter: flexible tube surgically inserted into the the intrathecal space at the lumbar (most common), thoracic, or cervical region and connected to the pump

Baclofen flows from the pump and out of the catheter, surrounding the spinal cord

o With a programmable pump, adjustments can be made in the dose, rate, and timing of baclofen infusions

o System has an alarm that signals low reservoir volume and low battery

Better spasticity control at 100x lower dose than oral doses Less systemic side effects than oral baclofen Doses administered either in regular boluses and/or by continuous infusion, and need

daily adjusting to clinical effect Average dose= 50-1200 micrograms/day Patients are very sensitive to drug delivery rate changes, leaving them susceptible to

overdose and withdrawal states that can be life threatening Complications related to infection, programming errors, or migration/fracture of

catheterd. Side effects/clinical presentation:

Acute baclofen overdose

o Symptoms depend on the dose of baclofen being deliveredo Symptoms may include: hypotension, bradycardia/tachycardia, hypotonia,

flaccid paralysis, somnolence, delirium, respiratory depression, seizures, and cardiac abnormalities

o Profound respiratory depression and coma may follow overdose, needing emergency resuscitation

o Differential diagnoses include sepsis, intracranial bleed, hypoglycemia, and electrolyte imbalance

Acute baclofen withdrawalo Associated with rebound excitation at all levels of the neuraxis that may not be

overcome by the administration of small dosages of oral or ITB or other GABA agonists

o Symptoms may present close to scheduled refill dates or may be from catheter-related problems, an infected pump, pump removal, empty reservoir volume, end of battery life, and iatrogenic programming error

o 1-3 day evolution of symptomso Most common symptoms: dystonia, fever/hyperthermia, tachycardia, agitation,

hypertensiono Symptoms may also include: spasticity (earliest symptom), itching, seizures,

hallucinations, delirium, delusions and paranoiao Life-threatening symptoms: malignant hyperthermia, autonomic instability,

rhabdomyolysis, disseminated intravascular coagulation, and multi-organ system failure

o Differential diagnosis: seizures, autonomic dysreflexia, neuroleptic malignant syndrome, malignant hyperthermia, or abdominal emergency.

e. Patient management: EMERGENT THERAPY Acute overdose:

o Life support measures: airway, ventilatory, and circulatory support o No antidoteo Neurosurgery and physical medicine and rehabilitation consultation to:

Interrogate and/or stop the pump Drain CSF by lumbar puncture or by pump access port Assess baclofen concentration in CSF Surgically repair or revise the system components

o Admit to intensive care unit, depending on clinical stability Acute withdrawal

o Life support measures: airway, ventilatory, and circulatory supporto High-dose oral* or enteral baclofen early in the clinical course

Adult dose: 120 mg/day in 6-8 divided doses *Oral baclofen should NOT be the sole treatment for baclofen

withdrawal as it does not achieve high enough CSF levels

o Neurosurgery and physical medicine and rehabilitation consultation to: Restore drug infusion at/near the pre-withdrawal dosage via the pump Administer ITB via a programmed bolus, through the catheter access

port, by lumbar puncture, or through an externalized cathetero IV Benzodiazepines

Continuous or intermittent infusion until ITB therapy is restored Titrate dosage until the desired therapeutic effect is achieved

o Dantrolene infusion may relieve muscle rigidity but does not reverse the other manifestations of GABAergic agonist withdrawal

o Monitor for rhabdomyolysis and disseminated intravascular coagulation, treat accordingly

o Admit to appropriate level of care, likely intensive care unitf. Patient management: DEVICE TROUBLESHOOTING

Consult a physician experienced in ITB therapy, preferably the physician managing the patient’s pump (i.e. Neurosurgery and Physical Medicine and Rehabilitation) to:

o Interrogate the ITB pump using the manufacturer’s programming deviceo Empty the pump reservoir, refill it with baclofen solution at the appropriate

concentrationo Determine the cause of ITB therapy interruptiono Surgically repair, revise, or replace system components

Radiologic exam of the pump and catheter system to identify mechanical disruptionso Xray abdomen (AP/Lateral) to evaluate catheter position and tubingo Catheterogram (fluoro) to assess continuity of tubing

References:1. Coffey RJ, Edgar TS, Francisco GE, Graziani V, Meythaler JM, Ridgely PM, Sadiq SA, Turner MS. Abrupt

withdrawal from intrathecal baclofen: recognition and management of a potentially life-threatening syndrome. Arch Phys Med Rehabil 2002, 83:735-741

2. Kao LW, Amin Y, Kirk MA, Turner MS. Intrathecal baclofen withdrawal mimicking sepsis. J Emerg Med 2003, 24:423-427

3. Haranhalli N, Anand D, Wisoff JH, Harter DH, Weiner HL, Blate M, Roth J. Childs Nerv Syst. 2011 Mar; 27 (3): 421-7. Doi: 10.1007/s00381-010-1277-9. Epub 2010 Sep 18. PMID: 20853002

4. Watve SV, Sivan M, Raza WA, Jamil FF. Management of acute overdose or withdrawal state in intrathecal baclofen therapy. Spinal Cord. 2012 Feb;50(2):107-11.