Complex Care Curriculum Autonomic Dysreflexia

Jimmy Beck, MD Rebekah Conroy, MD Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition 1)Define Autonomic Dysreflexia (AD) in

Pathophysiology simple terms Severe Complications 2)Describe the pathophysiology Precipitants

Clinical Manifestations 3)List the life threatening complications

Acute Management Algorithm 4)List the most common precipitants Initial Assessment

Non-pharmacologic 5)List signs and symptoms Treatment Pharmacologic Treatment 6)Formulate an acute management plan Summary using both non-pharmacologic and References pharmacologic treatment options Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives Definition Clinical phenomenon with life threatening potential Pathophysiology ∙ Patients with spinal cord injuries Severe Complications

Precipitants Location of injury Clinical Manifestations

Acute Management ∙ At over above T-6 Level Algorithm

Initial Assessment

Non-pharmacologic Incidence Treatment Pharmacologic ∙ 16% of children and adolescents Treatment

Summary Timeframe References ∙ 2-6 months after injury Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology

Severe Complications

Precipitants

Clinical Manifestations

Acute Management Algorithm

Initial Assessment Non-pharmacologic Noxious Treatment stimuli Pharmacologic Treatment

Summary

References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition Hemorrhage

Pathophysiology

Severe Complications Death Precipitants

Clinical Manifestations

Acute Management Cerebral Algorithm Initial Assessment

Non-pharmacologic Treatment Coma Pulmonary Pharmacologic Edema Treatment

Summary

References Arrhythmias

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives GENITOURINARY GASTROINTESTINAL Definition

Pathophysiology

Severe Complications

Precipitants

Clinical Manifestations

Acute Management Algorithm • Bladder distension • Bowel distension due to Initial Assessment fecal impaction • stones Bladder issues account for Non-pharmacologic • Blocked catheter • Appendicitis Treatment 75-85% of AD episodes. • Gallstones Pharmacologic • Catheterization Treatment • Instrumentation • Gastric ulcers or gastritis • Anal fissure Summary • Urinary tract infection • Enema or suppository References • Barium enema • Colonoscopy Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives DERMATOLOGY REPRO. SYSTEM Definition

Pathophysiology

Severe Complications

Precipitants

Clinical Manifestations

Acute Management Algorithm • Constrictive clothing • Labor and delivery Initial Assessment • Contact with sharp • Menstruation Non-pharmacologic objects • Intercourse Treatment • Blister Pharmacologic • Ejaculation Treatment • Burn • STD • Frostbite Summary • Scrotal compression • Ingrown toenail • Epididymitis References • Insect bite • Vaginitis • Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition MUSCULOSKELETAL Pathophysiology HEMATOLOGY MEDS

Severe Complications

Precipitants

Clinical Manifestations

Acute Management Algorithm

Initial Assessment • Pulmonary • Fracture • Excessive alcohol embolism • Trauma intake Non-pharmacologic • Excessive caffeine Treatment • Deep vein • Joint dislocation Pharmacologic thrombosis • Excessive diuretic Treatment intake • Nasal Summary decongestants References • Sympathomimetics

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition SYMPTOMS SIGNS Pathophysiology • Anxiety • Paroxsymal HTN Severe Complications • Pounding headache • Precipitants • Blurry vision • Ocular findings • • Fecal Impaction Clinical Manifestations • Metallic taste in mouth • Penile erection Acute Management Algorithm • Respiratory distress • Diaphoresis of forehead

Initial Assessment • Nausea • Blanching rash on face and neck and chest Non-pharmacologic • Urge to void Treatment • Increased spasticity • Cutis anserina Pharmacologic Treatment • Parasthesias

Summary

References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology

Severe Complications

Precipitants

Clinical Manifestations

Acute Management Algorithm

Initial Assessment

Non-pharmacologic Treatment Pharmacologic Treatment

Summary

References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives Assess patient: including Temp, HR, BP Definition

Pathophysiology

Severe Complications Sit patient upright Precipitants

Clinical Manifestations Acute Management Remove any tight clothing Algorithm

Initial Assessment

Non-pharmacologic Treatment Pharmacologic Recheck HR and BP every 5mins Treatment

Summary

References If febrile, remove any external temperature sources Drug Formulary

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology

Severe Complications Non-pharmacologic management will resolve the majority of AD episodes. Precipitants

Clinical Manifestations

Acute Management Algorithm

Initial Assessment Mild – Moderate HTN Non-pharmacologic Severe HTN • ≤ 13yrs: SBP ≥ 15-30 Treatment • ≤ 13yrs: SBP >30mmHg mmHg above baseline Pharmacologic above baseline Treatment • > 13yrs: SBP ≥ 20 mmHg • > 13yrs: SBP >150mmHg Summary above baseline up to 150mmHg References

Drug Formulary

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia Learning Objectives Mild to Moderate Definition Blood Pressure Elevation

Pathophysiology Perform bladder management steps Severe Complications

Precipitants Repeat vital signs Clinical Manifestations

Acute Management Algorithm If BP remains unchanged or Initial Assessment If BP decreases, monitor increases, notify MD Non-pharmacologic until VS normal & patient Treatment asymptomatic Pharmacologic Perform bowel management steps. Treatment Consider hypertension medications. Summary

References If blood pressure elevation

Drug Formulary becomes severe, transfer to ICU

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition Severe Blood Pressure Elevation

Pathophysiology

Severe Complications Notify MD

Precipitants Consider or nitropaste Clinical Manifestations

Acute Management Algorithm Perform bladder management steps*

Initial Assessment

Non-pharmacologic Place patient on monitor Treatment Pharmacologic Treatment If BP remains elevated, perform bowel management steps,* Summary and start hypertension medications if not previously done References

Drug Formulary If BP elevation remains severe, prepare for transfer to ICU

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition If Foley present If no Foley

Pathophysiology

Severe Complications Insert 2% xylocaine jelly into urethra. Wait 2 Remove any kinks minutes and insert Foley catheter using jelly as lubricant Precipitants

Clinical Manifestations

Acute Management If plugged, irrigate with 5-10 ml of sterile normal Avoid manually compressing or tapping on Algorithm saline for kids < 2 yrs and 10-15 ml for kids > 2 yrs bladder

Initial Assessment

Non-pharmacologic Treatment If remains plugged, remove it and insert 2% xylocaine jelly into urethra. Wait 2 minutes and Leave catheter in place if difficult catheterization Pharmacologic insert new foley catheter using jelly as lubricant Treatment

Summary

References Avoid manually compressing or tapping on bladder Drug Formulary

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives Insert 2% xylocaine jelly into rectum Definition

Pathophysiology Wait 2 minutes and examine rectum, Severe Complications checking for presence of stool

Precipitants

Clinical Manifestations Remove any stool if present

Acute Management Algorithm If autonomic dysreflexia does not resolve or Initial Assessment blood pressure continues to increase Non-pharmacologic Treatment Pharmacologic Stop manual evacuation and instill additional Treatment 2% xylocaine jelly Summary

References After 20 minutes, recheck for presence of stool and remove if present Drug Formulary

McGinnis et al. J Spinal Cord Med. 2004. Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition Nitropaste Pathophysiology

Severe Complications

Precipitants

Clinical Manifestations Acute Management Hydralazine Nifedipine Algorithm Long acting medications? are not Initial Assessment indicated in the management Non-pharmacologic of acute episodes. Treatment Pharmacologic Treatment

Summary References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology Severe Complications Nitroglycerin paste Precipitants

Clinical Manifestations

Acute Management Algorithm Rapid onset, reversible Initial Assessment

Non-pharmacologic Treatment Pharmacologic Venous pooling and the drop in BP may Treatment trigger an alpha agonist release thereby Summary exacerbating the AD References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology Severe Complications Nifedipine Precipitants

Clinical Manifestations

Acute Management Algorithm Rapid onset Initial Assessment

Non-pharmacologic Treatment Pharmacologic May cause headache, , Treatment dizziness, fatigue, nausea, or orthostatic Summary hypotension References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology Severe Complications Hydralazine Precipitants

Clinical Manifestations

Acute Management Algorithm Rapid onset, titratable Initial Assessment

Non-pharmacologic Treatment Pharmacologic May cause reflex tachycardia, prolonged Treatment hypotension, nausea. Summary Requires IV access References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition

Pathophysiology Severe Complications Clonidine Precipitants

Clinical Manifestations

Acute Management Algorithm Rapid onset with oral form Initial Assessment

Non-pharmacologic Treatment Pharmacologic Treatment Withdrawal can cause life threatening

Summary hypertension

References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives Definition Other Medications Pathophysiology • Phenoxybenzamine Severe Complications

Precipitants • Prazosin

Clinical Manifestations • Acute Management • Oxybutynin Algorithm Initial Assessment • Nitroprusside Non-pharmacologic Treatment • Diazoxide Pharmacologic Treatment • Trimethaphan camphorsulfonate Summary • Phentolamine References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition Pathophysiology Non-Pharmacologic Severe Complications • Identify and remove Precipitants the noxious stimuli Clinical Manifestations T6 • Bladder issues are the Acute Management most common trigger Algorithm followed by GI issues Initial Assessment Non-pharmacologic Pharmacologic Treatment Pharmacologic • Nitroglycergin paste Treatment

Summary • Nifedipine

References

Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives 1. Blackmer J. Rehabilitation medicine: 1. autonomic dysreflexia. CMAJ. 2003;169(9):931-935. Definition

Pathophysiology 2. Braddom RL. Physical medicine & rehabilitation. 3rd ed. ; 2006:632- 633. Severe Complications

Precipitants 3. Braddom RL, Rocco JF. Autonomic dysreflexia. A survey of current treatment. Am J Phys Med Rehabil. 1991;70(5):234-241. Clinical Manifestations

Acute Management 4. Consortium for Spinal Cord Medicine. Acute management of Algorithm autonomic dysreflexia: Individuals with presenting to J Spinal Cord Med Initial Assessment health-care facilities. . 2002;25 Suppl 1:S67-88.

Non-pharmacologic Treatment 5. Erickson RP. Autonomic : Pathophysiology and medical Pharmacologic management. Arch Phys Med Rehabil. 1980;61(10):431-440. Treatment

Summary 6. Karlsson AK. Autonomic dysreflexia. Spinal Cord. 1999;37(6):383-391.

References

Drug Formulary References Continued  Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives 7. Krassioukov A, Warburton DE, Teasell R, Eng JJ, Spinal Cord Injury Rehabilitation Evidence Research Team. A systematic review of the Definition management of autonomic dysreflexia after spinal cord injury. Arch Phys Pathophysiology Med Rehabil. 2009;90(4):682-695. doi: 10.1016/j.apmr.2008.10.017; 10.1016/j.apmr.2008.10.017. Severe Complications 8. Lee BY, Karmakar MG, Herz BL, Sturgill RA. Autonomic dysreflexia Precipitants revisited. J Spinal Cord Med. 1995;18(2):75-87.

Clinical Manifestations 9. Lindan R, Joiner E, Freehafer AA, Hazel C. Incidence and clinical Acute Management features of autonomic dysreflexia in patients with spinal cord injury. Algorithm Paraplegia. 1980;18(5):285-292. doi: 10.1038/sc.1980.51. Initial Assessment Non-pharmacologic 10. McGinnis KB, Vogel LC, McDonald CM, et al. Recognition and Treatment management of autonomic dysreflexia in pediatric spinal cord injury. J Pharmacologic Spinal Cord Med. 2004;27 Suppl 1:S61-74. Treatment Summary 11. Naftchi NE, Richardson JS. Autonomic dysreflexia: Pharmacological References management of hypertensive crises in spinal cord injured patients. J Spinal Cord Med. 1997;20(3):355-360. Drug Formulary Complex Care Curriculum Autonomic Dysreflexia

Please take note of the following prior to using this formulary:

 Most of these medications are not FDA approved for the treatment of , particularly in the pediatric population.

 The dosage information provider here is a combination of recommendations from the Pediatric Dosing Handbook Formulary and expert opinion.

 It is important for this dosing information to be used with caution and only after full review of the entire dysautonomia module.

 We highly encourage you to discuss medication dosing with a pharmacist and/or pediatric physiatrist if you have any questions or concerns.

Continue to Drug Formulary Complex Care Curriculum Autonomic Dysreflexia Learning Objectives Nitroglycerin Definition Mechanism of Direct acting vasodilator; causes dilation of the Pathophysiology Action venous system; decreases preload of the heart Severe Complications Dose •Nitroglycerin 2% paste: Start with 0.5 inch and Precipitants increase by 0.5 inch increments to achieve desired results. Apply topically 1 inch above the spinal cord Clinical Manifestations injury level (max of 2 doses/day) •Sublingual (adults): 0.3-0.6 mg q5 minutes (max of 3 Acute Management Algorithm doses in 15 minutes) Initial Assessment Onset 10-30 minutes Non-pharmacologic 3-6 hours; may wipe away paste to stop action Treatment Duration Pharmacologic Treatment Precautions May aggravate AD by reflexively increasing sympathetic alpha agonist release secondary to Summary venous pooling and drop in BP References Dosage Forms Topical: 2% ointment Drug Formulary SL: 0.3 mg, 0.4 mg, 0.6 mg tablets Nifedipine Nitroglycerin Nifedipine Hydralazine Clonidine Capsule Complex Care Curriculum Autonomic Dysreflexia Learning Objectives Nifedipine Definition ; potent coronary and Pathophysiology Mechanism of Action peripheral arterial vasodilator Severe Complications Dose Children: 0.25-0.5mg/kg/dose (max 10 mg), Precipitants may repeat every 4-6 hours as needed

Clinical Manifestations Adult: 10mg chewable Acute Management Onset SL/“bite and swallow”: within 1 – 5 minutes Algorithm IR: within 20 – 30 minutes Initial Assessment Duration 4 – 8 hours Non-pharmacologic Treatment May cause headache, tachycardia, dizziness, Pharmacologic Precautions Treatment fatigue, nausea, or . Ineffective at prevention of episodes. Do not Summary use the sustained release form. References Dosage Forms 10 mg capsule (contains 10 mg/0.34 mL); liquid Drug Formulary must be withdrawn from capsule Nifedipine Nitroglycerin Nifedipine Hydralazine Clonidine Capsule Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives Definition Nifedipine Capsule: Pathophysiology 10 mg contains 0.34 mL Severe Complications

Precipitants Weight (Kg) Dose Range Volume

Clinical Manifestations ≥ 10 2.5 mg – 5 mg 0.09 ml – 0.17 ml Acute Management Acute Management ≥ 15 3.75 mg – 7.5 mg 0.13 ml – 0.26 ml Algorithm Algorithm ≥ 20 5 mg – 10 mg 0.17 ml – 0.34 ml Initial Assessment Initial Assessment Non-pharmacologic ≥ 30 7.5 mg – 10 mg 0.26 ml – 0.34 ml Non-pharmacologic Treatment Treatment ≥ 40 10 mg 0.34 ml Pharmacologic Pharmacologic Treatment Treatment Summary Summary References References & Formulary Drug Formulary McGinnis et al. J Spinal Cord Med. 2004. Nifedipine Nitroglycerin Nifedipine Hydralazine Clonidine Capsule Complex Care Curriculum Autonomic Dysreflexia

Learning Objectives

Definition Hydralazine Relaxes smooth muscle; causes peripheral Pathophysiology Mechanism of . Severe Complications Action IV push: 0.1-0.2 mg/kg/dose IM/IV every 4-6 Precipitants Dose hours as needed (max dose 20mg/dose). Clinical Manifestations 5 – 20 minutes Acute Management Onset Algorithm Duration 2 – 6 hours Initial Assessment May cause tachycardia, vomiting, and Non-pharmacologic Precautions Treatment headache. May give with diuretic and a beta- Pharmacologic blocker to counteract side effects of sodium Treatment and water retention and reflex tachycardia. Summary Concentration 20 mg/mL References

Drug Formulary Nifedipine Nitroglycerin Nifedipine Hydralazine Clonidine Capsule Complex Care Curriculum Autonomic Dysreflexia Learning Objectives Clonidine Definition Mechanism of Alpha-2 agonist; decreases sympathetic outflow from Pathophysiology Action CNS; peripheral vascular resistance, heart rate, blood pressure, and renal vascular resistance. Severe Complications

Precipitants Dose 1-17 years old: 0.05 – 0.1 mg/dose (may repeat Clinical Manifestations hourly up to max total dose 0.8 mg) < 1 year old: not recommended (could use dose of Acute Management Algorithm 2.5 mcg/kg/dose if no other options)

Initial Assessment Onset 30 – 60 minutes Non-pharmacologic Duration 6 – 10 hours Treatment Pharmacologic Precautions Serious bradycardia may occur if given with other Treatment sympatholytic drugs. Rebound hypertension may Summary develop with abrupt withdrawal. May also cause sedation and CNS side effects. References 0.1 mg tablet Drug Formulary Dosage Forms Nifedipine Nitroglycerin Nifedipine Hydralazine Clonidine Capsule