Irukandji SyndromeIsabel M. Algaze Gonzalez, MD

Assistant Professor of Emergency Medicine

Wilderness Medicine Fellowship Co-Director

University of California, Irvine

Physician at Catalina Island Medical Center

Avalon, Catalina

Funded by: U54MD008149RTRN, NIMHDPI Angel Yanagihara

Objectives

• Brief bio-chemical review

• Pathophysiology

• Clinical presentation

• Most recent treatment strategies

No disclosures

Why is this important to you? -Goals

• You can not diagnose what, to your knowledge, does not exist

• Increasingly prevalent around the world coastal areas

• Need to prevent:• Misdiagnosis

• Underreporting

• Improper treatment strategies

Why is this important to you? -Goals

• Has a great burden of morbidity and mortality

• Associated financial costs are high

Credit: Dianna Emmanuelli

Jellyfish Background Information

Distribution

Alatina alata Nematocyst

Sting Mechanism

What do we know

• Severity increases with:

• Dose• Jellyfish size

• Extent of contact

• Delicacy of the victim's skin

• Fish have nucleated cells• Capability to repair makes them 1,000 times more resistant than humans

• Nematocysts Injects Spines • Mineralized Chitin- Triggers Mannose protein mediated prolonged immune response

• Porin • Approximately 1% if the venom

• Causes holes in cells

• Platelets are affected first• Releases catecholamines

• Anthrax porin (anthrolysin O) as a model• Copper (better than zinc) Gluconate inhibits Calcium mediated porin assembly

• Death • Marker of death is potassium

• After well intentioned epinephrine

King Slayer, Carukia

barnesi, Malo kingi

Chironex fleckeri

Literature

Pathophysiology of cubozoan envenomation

Clinical presentation

• 1 to 2 hours after the sting• May not have cutaneous marks

• Cutaneous:• May progress over a course of days to local necrosis, skin

ulceration, and secondary infection• Can resolve 2w-2mo• Residual hyperpigmentation to lichenification

• Ocular: • Conjunctivitis, chemosis, corneal ulcers, corneal

epithelial edema, keratitis, iridocyclitis, elevated intraocular pressure, synechiae, iris depigmentation, chronic unilateral glaucoma, and lacrimation

• Systemic:• “Feeling of doom”, Trembling, Acute renal failure,

lymphadenopathy, chills, fever, and nightmares

Credit: Dianna Emmanuelli

Clinical presentation

• Neurologic: • Malaise, headache, aphonia, diminished touch and temperature sensation, vertigo, ataxia, spastic or

flaccid paralysis, mononeuritis multiplex, Guillain-Barré syndrome, parasympathetic dysautonomia, plexopathy, radial–ulnar–median nerve palsies, brainstem infarction, delirium, loss of consciousness, convulsions, coma, and death

• Cardiovascular: • Hemolysis, hypotension, small artery spasm, bradyarrhythmias, tachyarrhythmias, elevated serum

troponin I level in the absence of myocardial injury, vascular spasm, deep venous thrombosis, thrombophlebitis, acute myocardial infarction, congestive heart failure, and ventricular fibrillation

• Respiratory: • Rhinitis, bronchospasm, laryngeal edema, dyspnea, cyanosis, pulmonary edema, and respiratory failure

• Musculoskeletal or rheumatologic: • Abdominal rigidity, diffuse myalgia and muscle cramps, muscle spasm, fat atrophy, arthralgias, reactive

arthritis, and thoracolumbar pain

• Gastrointestinal: • Nausea, vomiting, diarrhea, paralytic ileus, dysphagia, hypersalivation, and thirst

Treatment

Clinical trial and piglet model

• Collaboration • DOD

• Puerto Rico Sea Grant

• Hawaii Sea Grant

• NIH grant

Treatment

• ACLS/BLS protocols acutely

• Anticipate drowning

• Hypothermia

• Decompression sickness or arterial air embolism

• If only local symptoms:

• Treat symptomatically-• Vinegar and then hot water

• Sting no More products

• Observe until the victim is asymptomatic for 6 hours*

• Antivenom vs inhibitor • Technical limitations

Treatment

• What NOT to do:

• Rinse with fresh water

• Rinse with cold water

• Apply ice

• Scrape with a credit card

• Shave with shaving cream

• Rub sand

• Rinse with urine

• Rinse with alcoholic drinks or rubbing alcohol

• Touch with bare hands

• Administer Epinephrine

Literature

Literature

Without blood skin agar With skin blood agar

Prevention

• Beach monitoring

• Flag system and beach signs

• Bay Watch and EMS most have updated protocols

• Monitoring of victims*

• Poison center reporting

Credit: Dianna Emmanuelli

Management

• If symptomatic:

• Monitor- some end up admitted to ICU

• Opioids – spasm pains, careful of pulmonary edema

• Nausea medication

• Phentolamine- Alpha blockers have been recommended for the control of hypertension, as a result of catecholamine release.

• Mag- for arrhythmias?

Draft by Irukandji clinical working group

You have an expert in your backyard

Please forward cases and pictures to :

Angel A. Yanagihara, Ph.D.

Associate Research Professor

Bekesy Laboratory of Neurobiology, PBRC

and Dept of Trop Med, JABSOM

University of Hawaii at Manoa

1993 East West Road

Honolulu, Hawaii 96822 USA

Email ayanagih@hawaii.edu

Tel 808 956-8328

FAX 808 956-8713

http://manoa-hawaii.academia.edu/AngelYanagihara

https://www.researchgate.net/profile/Angel_Yanagihara

Isabel M. Algaze Gonzalez, MD

Assistant Professor of Emergency Medicine

Wilderness Medicine Fellowship Co-Director

University of California, Irvine

333 City Boulevard West, 640, Orange, CA, 92868

Physician at Catalina Island Medical Center

Avalon, Catalina

100 Falls Canyon Rd, Avalon, CA 90704

Email ialgazeg@uci.edu

Tel 714 456-3713

FAX 714-456-3714

http://www.ucirvinehealth.org/find-a-doctor/a/isabel-m-algaze-gonzalez

For the future

• More research is needed on

• Species

• Distribution

• Syndrome

• Variations in signs and symptoms between species

• Validated Treatment

• Prevention strategies

References• TJ. Carrette, AH. Underwood, JE. Seymour, “Irukandji Syndrome: a Widely Misunderstood and Poorly Researched Tropical Marine Envenoming,” Diving and Hyperbaric Medicine : the Journal of the

South Pacific Underwater Medicine Society, Vol. 42, No. 4, 2012, pp. 214-23.

• PJ. Fenner, “Dangers in the Ocean: the Traveler and Marine Envenomation. I. Jellyfish,” Journal of Ttravel Medicine, Vol. 5, No. 3, 1998, pp. 135-41.

• J. Tibballs, R. Li, HA. Tibballs, LA. Gershwin, KD. Winkel, “Australian Carybdeid Jellyfish Causing "Irukandji Syndrome",” Toxicon, Vol. 9, No. 6, 2012, pp. 617-25. doi:10.1016/j.toxicon.2012.01.006

• L. Goggin, LA. Gershwin, P. Fenner, J. Seymour, T. Carrette, “Stinging Jellyfish in Tropical Australia: Current State of knowledge,” Cooperative Research Center Reef Research Centre Brochure, 2004

• D. Calder, “Cubozoan and Scyphozoan Jellyfishes of the Carolinian Biogeographic Province, Southeastern USA,” Royal Ontario Museum: Contributions in Science Brochure, Vol. 3, 2009. ISBN: 978-0-88854-471-1

• M. Little, P. Pereira, T. Carrette, J. Seymour, “Jellyfish Responsible for Irukandji Syndrome,” QJM : Monthly Journal of the Association of Physicians, Vol. 99, No. 6, 2006, pp. 425-7.

• L. Gershwin, “Jellyfish Responsible for Irukandji Syndrome,” QJM : Monthly Journal of the Association of Physicians, Vol. 99, No.11, 2006, pp. 801-2; author reply 803-4.

• B. Bentlage, P. Cartwright, AA. Yanagihara, C. Lewis, GS. Richards, AG. Collins, “Evolution of Box Jellyfish (Cnidaria: Cubozoa), a Group of Highly Toxic Invertebrates,” Proceedings of the The Royal Society: Biological Sciences, Vol. 277, No. 1680, 2010, pp. 493-501. doi: 10.1098/rspb.2009.1707

• PM. Bailey, M. Little, GA. Jelinek, JA. Wilce, “Jellyfish Envenoming Syndromes: Unknown Toxic Mechanisms and Unproven Therapies,” The Medical Journal of Australia, Vol. 178, No. 1, 2003, pp. 34-7.

• PJ. Fenner, JC. Hadok, “Fatal Envenomation by Jellyfish Causing Irukandji Syndrome,” The Medical Journal of Australia, Vol. 17, No. 7, 2002, pp. 362-3.

• JH. Barnes, “Cause and Effect in Irukandji Stingins,” The Medical Journal of Australia, Vol. 1, 1964, pp. 897-904.

• TT. Huynh, J. Seymour, P. Pereira, R. Mulcahy, P. Cullen, T. Carrette, M. Little, “Severity of Irukandji Syndrome and Nematocyst Identification from Skin Scrapings,” The Medical Journal of Australia, Vol. 178, No. 1, 2003, pp. 38-41.

• K. Tiong, “Irukandji Syndrome, Catecholamines, and Mid-Ventricular Stress Cardiomyopathy,” European Journal of Echocardiography, Vol. 10, No. 2, 2009, pp. 334-6. doi:10.1093/ejechocard/jen246

• D. McD Taylor, P. Pereira, J. Seymour, KD. Winkel, “A Sting from an Unknown Jellyfish Species Associated with Persistent Symptoms and Raised Troponin I Levels,” Emergency Medicine (Fremantle, WA), Vol. 14, No. 2, 2002, pp. 175-80.

References• P. Pommier, M. Coulange, L. De Haro, “Systemic Envenomation by Jellyfish in Guadeloupe: Irukandji-Like Syndrome?” Médecine Tropicale: Revue du Corps de Santé Colonial, Vol. 65, No. 4, 2005, pp.

367-9.

• CP. Nickson, EB. Waugh, SP. Jacups, BJ. Currie, “Irukandji Syndrome Case Series from Australia's Tropical Northern Territory,” Annals of Emergency Medicine, Vol. 54, No. 3, 2009, pp. 395-403. doi: 10.1016/j.annemergmed.2009.03.022

• L. Domachevsky. L. Rachmany, Y. Barak, V. Rubovitch, A. Abramovich, CG. Pick, “Hyperbaric Oxygen-Induced Seizures Cause a Transient Decrement in Cognitive Function,” Neuroscience, Vol. 247, 2013, pp. 328-34. doi:10.1016/j.neuroscience.2013.05.052

• ES. Shank, CM. Muth, “Diver with Decompression Injury, Elevation of Serum Transaminase Levels, and Rhabdomyolysis,” Annals of Emergency Medicine, Vol. 37, No. 5, 2001, pp. 533-6.

• R. Courtney, J. Seymour, “Seasonality in Polyps of a Tropical Cubozoan: A latina nr mordens,” PLoS One, Vol. 8, No. 7, 2013; e69369. doi: 10.1371/journal.pone. 0069369

• GC. Gray, VJ. Martin, RA. Satterlie, “UltraStructure of the Retinal Synapses in Cubozoans,” The Biological Bulletin, Vol. 217, No. 1, 2009, pp. 35-49.

• NJ. Cohen, et al., “Preventing Maritime Transfer of Toxigenic Vibrio cholera” Emergent Infectious Diseases, Vol. 18, No. 10, 2012, pp. 1680–1682. doi: 10.3201/ eid1810.120676

• P. Miloslavich, et al., “Marine Biodiversity in the Caribbean: Regional Estimates and Distribution Patterns”, PLoS One. Vol. 5, No. 8, 2010, e11916. doi: 10.1371/journal.pone.0011916

• M. David, S. Gollasch, E. Leppäkoski, “Risk Assessment for Exemptions from Ballast Water Management - The Baltic Sea Case Study,” Marine Pollution Bulletin, 2013. doi: 10.1016/j.marpolbul.2013.07.031

• GT. Taylor, et al., “Ecosystem Responses in the Southern Caribbean Sea to Global Climate Change,” Proceedings of the National Academy of Sciences, Vol. 109, No. 47, 2012, pp. 19315–19320. doi: 10.1073/ pnas.1207514109

• DA. Warrell, “Venomous Bites, Stings, and Poisoning,” Infectious Disease Clinics of North America, Vol. 26, No. 2, 2012, pp. 207-23. doi: 10.1016/j.idc.2012.03.006

• J. Rathbone, J. Quinn, S. Rashford, “Response to 'Randomised Trial of Magnesium in the Treatment of Irukandji Syndrome'.” Emergency Medicine Australas, Vol. 25, No. 1, 2013, pp. 97-98. doi:10.1111/ 1742-6723.12026

• N. McCullagh, P. Pereira, P. Cullen, R. Mulcahy, R. Bonin, M. Little, S. Gray, J. Seymour, “Randomised Trial of Magnesium in the Treatment of Irukandji Syndrome.” Emergency Medicine Australas, Vol. 24, No. 4, 2012, pp. 560-5. doi:10.1111/j.1742-6723.2012.01602.x