Objectives MANAGEMENT OF DYSTONIA AND BREAKTHROUGH SEIZURES: THE ROLE OF PALLIATIVE CARE TEAMS. Neurological Impairment (NI) 10/24/18

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1 MANAGEMENT OF DYSTONIA AND BREAKTHROUGH SEIZURES: THE ROLE OF PALLIATIVE CARE TEAMS Julie Hauer, MD Maruzza Congress 24 October 2018 Objectives Appreciate central pain as a trigger for severe movement and posturing (Attitude) Discuss treatment options for dystonia (Knowledge) Practice developing a home based plan for prolonged seizures (Skill) Neurological Impairment (NI) Indicated by developmental label cerebral palsy A result of a CNS insult hypoxic ischemic encephalopathy (HIE), anoxic encephalopathy, traumatic brain injury (TBI) Associated with a specific diagnosis genetic disorder, congenital anomaly, structural brain malformation, or metabolic disorder 1

2 Neurological Symptoms Neurological impairment = NI Seizures Spasticity Autonomic dysfunction Dystonia Central neuropathic pain Basal ganglia: control of movement (Dystonia) Thalmus: somatosensory transmission (Central Pain) Hypothalamus: regulation of heart rate, temperature, blood pressure (Dysautonomia) Cerebrum: decreased inhibition, spasticity Medulla: vasomotor and vomiting centers General Principles Different neurological problems and symptoms have similar features Children with global CNS impairment: if 1 exists, risk for others to coexist Central neuropathic pain can worsen any neurological symptom 2

3 CNS Problems Seizures Spasticity/ Muscle spasms Autonomic dysfunction/ PAID Dystonia Central neuropathic pain Hauer 2012, 2015 Anti-seizure drugs Benzodiazepines Baclofen Dantrolene Alpha-2 agonists Gabapentinoids Opioids Tricyclic (TCA) Beta blockers Trihexyphenidyl Antipsychotics Cannabinoids Dystonia Involuntary muscle contractions with slow twisting and repetitive movements, abnormal postures, or both Can result in pain Pain from other sources can worsen dystonic movement Primary versus Secondary Diagnosis versus a label Primary Dystonia Secondary dystonia o Global impairment, microcephaly o Risk for co-existing problems (spasticity, seizures, dysautonomia, central neuropathic pain) 3

4 Treatment: Goals Maximize function Decrease pain Enhance ease of care-giving Interdisciplinary team: o Therapists, Neurologists, PM&R o Palliative medicine (severe NI) Treatment: Medications General indications o First line Baclofen o Second line Trihexiphenidyl Painful dystonia o Gabapentin Dystonia with disturbed sleep o Clonidine 4

5 Empirical Treatment Anticholinergics: --Trihexyphenidyl --Benztropine Dopamine depletor --Tetrabenazine Gabapentin Baclofen L-Dopa Dystonia Dystonia, Chorea Neuropathic pain Dystonia, Spasticity Dopamine responsive Treatment: Surgical Intrathecal baclofen (ITB) pump Deep brain stimulator (DBS) Dystonia with pain behaviors: drug trials before surgical interventions Medications for dystonia and pain Gabapentinoid + Tricyclic antidepressant Gabapentinoid + Clonidine Case Dystonia 3 year old with hypoxic/hypotensive event, MRI basal ganglia Severe dystonic movement and irritability daily Meds: clonazepam, lorazepam gabapentin, methadone, baclofen (trihexyphenidyl stopped due to side effects) 5

6 Before Next Intervention Consider nociceptive sources Maximize drug doses Frequency/duration of episodes Effectiveness of breakthrough symptom care plan Manage possible triggers Hauer J, Houtrow A, AAP clinical report, June 2017 Integrative Management Rocking, massage, repositioning, fan, cool air, music, water, aromatherapy Vibratory stimulation (mats, pillows) Supportive equipment (seating, pillows) Calm environment Sleep, constipation Drug Problems treated Patient mg/kg/day (14.6 kg) Typical dose mg/kg/day Baclofen Spasticity * Clonazepam Lorazepam Gabapentin Dystonia, Spasticity Dystonia, Spasticity Autonomic dysfunction, Neuropathic pain, Spasticity ** *Lubsch 2006, **Korn-Merker

7 Case Dystonia Acute illness Increase in movement and agitation Risk for status dystonicus Acute care plan Home Hospital Status Dystonicus Increasingly frequent or continuous severe episodes of generalized dystonic spasms Trigger often identified Infection, medication toxicity or withdrawal, pain, constipation, puberty, ITB pump failure Home Care Plan Presenting features: grimacing, arching, twisting movement, stiffening Routine interventions: reposition, personal care Non-pharmacologic strategies: GI tract distention as a trigger: asneeded supp or enema, vent G-tube As-needed medications: ibuprofen, morphine, clonidine, or benzodiazepine Hauer and Houtrow 2017; Hauer

8 Hospital Care Plan Rhabdomyolysis, Respiratory Medications Clonidine starting dose mg/kg/ dose every 6 hours Midazolam continuous infusion References Dystonia AACPDM Dystonia in Cerebral Palsy Care Pathway, updated June 4, Allen NM, Lin JP, Lynch T, King MD. Status dystonicus: a practice guide. Dev Med Child Neurol. 2014;56(2): Buck ML. Trihexyphenidyl for the Management of Dystonia in Children. June /2015/12/ pdf Fehlings D, et al. Pharmacological and neurosurgical interventions for managing dystonia in cerebral palsy: a systematic review. Dev Med Child Neurol. 2018;60(4): Hauer J, Houtrow A. Pain Assessment and Treatment in Children With Significant Impairment of the Central Nervous System. Pediatrics. 2017;139(6):e References Dystonia Hauer JM. Neuropathic Pain as Potential Source of Feed-induced Dystonia in Children With Severe Central Nervous System Disorders. J Pediatr Gastroenterol Nutr. 2018;66(1):e25. Liow NY, et al. Gabapentin can significantly improve dystonia severity and quality of life in children. Eur J Paediatr Neurol. 2016;20(1): Lumsden DE, Kaminska M, Tomlin S, Lin JP. Medication use in childhood dystonia. Eur J Paediatr Neurol. 2016;20(4): Miguel R, et al. Tetrabenazine in treatment of hyperkinetic movement disorders: an observational study. Ther Adv Neurol Disord. 2017;10(2): Mordekar SR, Velayudhan M, Campbell DI. Feed-induced Dystonias in Children With Severe Central Nervous System Disorders. J Pediatr Gastroenterol Nutr. 2017;65(3):

9 Barry 15 year old, intractable epilepsy, Bipap, inpatient every 1-2 months past 6 months, hospitalization with mechanical ventilation Far from his baseline Goals: comfort, quality of life, intubate if reasonable chance for recovery 3 months later: 3 hospitalizations in 6-weeks for prolonged seizures Hypothetical Trajectory HEALTH, FUNCTION, QUALITY OF LIFE Fixable, Modifiable, or Irreversible TIME Improve Progressive Preserving health Re-goaling Preserving Comfort Intractable: not easily relieved or cured o Test and Fix Fracture Renal stones Bladder infection Respiratory infection o Modify with risk for intractable Seizures Dysautonomia GI motility Central pain Mucus clearance 9

10 Barry: 15 year old, intractable seizures I wish he could be seizure free, I worry What we may not want to do: It might make sense not to use (intubation, IVs) given that it doesn t improve his (seizures, GI), what are your thoughts...? What we can do: Offering options o Location of Care o Non-invasive management (home or hospital) o Use at home, Trial in the hospital Home Treatment Plan Diazepam rectal Lorazepam 0.1mg/kg (4mg) buccal/rectal Midazolam 0.2mg/kg (10mg) buccal/intranasal Rectal AEDs: carbamazepine, phenobarbital, lamotringine, and valproic Pediatric Pain and Symptom Management Guidelines 2014 Hauer, Duncan at %20Symptom%20Management%20Guidelines%20-%202014_ pdf Home Treatment Plan For seizure that persists for 5 minutes, give Diatat 25 mg rectal Lorazepam 4 mg buccal For seizure that persists another 10 minutes (20 min total) give lorazepam 4 mg buccal, may repeat 10 minutes later (30 min total) 4 hours after last dose of lorazepam: Lorazepam 2 mg every 8 hours VGT x 3, then 1 mg every 8 hours VGT x 3, then 1 mg every 12 hours x 2, then stop 10

11 Barry: 15 year old, intractable seizures 6 months later: Bipap discontinued 1 ½ years later, acute ileus: One option is to send him to the hospital to place an IV. I think the best plan is to use a trial of pedialyte by feeding tube. Thank you! Keep in touch! julie.hauer@childrens.harvard.edu 11