Epilepsy and Tuberous Sclerosis

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Focuses on Epilepsy Management for poeple wth Tuberous Sclerosis. Includes information on:
- Status Epilepticus
- Epilepsy Management
- First Aid Principles
- Seizure Management Planning

Epilepsy and Tuberous Sclerosis

  1. 2. Advances in Tuberous Sclerosis: From Pathway to Therapy Medical and Family Conference 3 – 4 November 2007 Sydney Children’s Hospital Randwick For more information: www.atss.org.au
  2. 3. I want to show you how it feels to have a seizure Hannah Age 9 157-6/05
  3. 4. <ul><li>Confident living, despite seizures </li></ul>Vision
  4. 5. <ul><li>Epilepsy Action delivers innovative, high quality education and support services across Australia </li></ul><ul><li>to minimise the impact of epilepsy and other </li></ul><ul><li>seizure disorders. </li></ul>Mission
  5. 6. <ul><li>Information and Referral Service </li></ul><ul><li>Individual Service Programs </li></ul><ul><li>Professional Education Programs </li></ul><ul><li>Community Education Programs </li></ul><ul><li>Consultancy </li></ul>Services
  6. 7. “ Epilepsy is the most common serious neurological disorder affecting an estimated 50 million people worldwide” (WHO) Epilepsy
  7. 8. <ul><li>1-2% of Australians have epilepsy (ABS). </li></ul><ul><li>5% of Australians will have a seizure during their lifetime (WHO ). </li></ul><ul><li>Approximately 80% of people with TSC will develop epilepsy </li></ul>Epilepsy
  8. 9. <ul><li>Epilepsy is a condition of recurrent, usually unprovoked, seizures often requiring medical management </li></ul>Epilepsy – What is it?
  9. 10. 36% 14% 7% 23% 6% 3% 8% 3% Seizure types Seizure Types
  10. 11. Seizure Classification Generalized Seizure involves whole brain & consciousness is affected Complex Altered awareness and behaviour Tonic Clonic “ grand-mal” or convulsion Atonic / Tonic “ drop attack” Myoclonic Sudden muscle jerks Absence “ petit mal” or staring fit Partial Seizure activity starts in one area of the brain Simple Retains awareness
  11. 12. Secondary generalization (spreading from one area to the whole brain) Complex Altered awareness and behaviour Partial Seizure activity starts in one area of the brain Simple Retains awareness Seizure Classification
  12. 13. Tonic Clonic “ grand-mal” or convulsion Atonic / Tonic “ drop attack” Myoclonic Sudden muscle jerks Absence “ petit mal” or staring fit Generalized Seizure involves whole brain & consciousness is affected Seizure Classification
  13. 14. <ul><li>Most Commonly associated with TSC </li></ul><ul><ul><li>West Syndrome </li></ul></ul><ul><ul><li>Lennox Gastaut </li></ul></ul>Epilepsy Syndromes
  14. 15. Status Epilepticus “ Any seizure lasting for a duration of at least 30 minutes or longer from which a person does not regain consciousness” Appleton and Gibbs 1998
  15. 16. Convulsive Status Epilepticus Easily recognised medical emergency requiring aggressive treatment
  16. 17. Non-convulsive Status Epilepticus Subtle, under recognised, not considered to be life threatening Walker 2004
  17. 18. Non-convulsive Status Epilepticus Simple partial status epilepticus Complex partial status epilepticus Absence status epilepticus Generalised myoclonic status
  18. 19. Non-convulsive Status Epilepticus <ul><li>Clinical indicators </li></ul><ul><ul><ul><li>Often subtle, long duration </li></ul></ul></ul><ul><ul><ul><li>Variation in responsiveness </li></ul></ul></ul><ul><ul><ul><li>Fluctuation in behaviour </li></ul></ul></ul><ul><ul><ul><li>Repetitive actions </li></ul></ul></ul><ul><ul><ul><li>Involuntary movements </li></ul></ul></ul><ul><ul><ul><li>Alteration in speech </li></ul></ul></ul>
  19. 20. Non-convulsive Status Epilepticus <ul><li>Treatment </li></ul><ul><ul><ul><li>Clinical diagnosis </li></ul></ul></ul><ul><ul><ul><li>EEG confirmation </li></ul></ul></ul><ul><ul><ul><li>Medicate </li></ul></ul></ul><ul><ul><ul><li>Monitor </li></ul></ul></ul>
  20. 21. Epilepsy Management <ul><li>Epilepsy diagnosis </li></ul><ul><li>Treatment options </li></ul><ul><ul><ul><li>Medication </li></ul></ul></ul><ul><ul><ul><li>Surgery </li></ul></ul></ul><ul><ul><ul><li>VNS </li></ul></ul></ul><ul><ul><ul><li>Ketogenic diet </li></ul></ul></ul><ul><ul><ul><li>Alternative therapies </li></ul></ul></ul><ul><li>Ongoing monitoring </li></ul>
  21. 22. Taking control - What You Can Do <ul><li>Knowledge </li></ul><ul><li>Working Partnerships </li></ul><ul><li>Accurate description </li></ul><ul><li>Seizure diary </li></ul><ul><li>First aid </li></ul><ul><li>ISP/SMP </li></ul><ul><li>Emergency medications </li></ul><ul><li>Peer support </li></ul>
  22. 23. Taking control - Knowledge <ul><li>Medical practitioner </li></ul><ul><li>Reputable websites </li></ul><ul><ul><ul><li>Major teaching hospital or university </li></ul></ul></ul><ul><ul><ul><li>Peak bodies </li></ul></ul></ul><ul><ul><ul><li>Professional body publications </li></ul></ul></ul><ul><ul><ul><li>Government organisations </li></ul></ul></ul><ul><li>Support organisations </li></ul><ul><ul><ul><li>National </li></ul></ul></ul><ul><ul><ul><li>International </li></ul></ul></ul><ul><li>Libraries </li></ul>
  23. 24. Taking control - Working Partnerships <ul><li>Medical practitioner </li></ul><ul><li>Allied health professionals </li></ul><ul><li>Early Intervention Unit </li></ul><ul><li>Teaching staff </li></ul><ul><li>Respite, day or accommodation service manager & staff </li></ul><ul><li>Employer </li></ul><ul><li>Recreational groups </li></ul>
  24. 25. Taking control – Description <ul><li>What was the person doing immediately prior to the seizure? </li></ul><ul><li>What called your attention to the seizure? </li></ul><ul><li>What did the person do during the seizure ? </li></ul>
  25. 26. Taking control – Description <ul><li>How long did the seizure last? </li></ul><ul><li>How did the person appear after the seizure ? </li></ul><ul><li> </li></ul><ul><li>How long before the person became orientated and responsive? </li></ul><ul><li>Any memory of the event? </li></ul>
  26. 27. <ul><li>Protect from injury </li></ul><ul><li>Time seizure activity </li></ul><ul><li>Summon help </li></ul>Taking control - First Aid Principles
  27. 28. <ul><li>Complex Partial Seizures </li></ul><ul><ul><li>Protect from injury </li></ul></ul><ul><ul><li>DO NOT restrain the person unless in danger </li></ul></ul><ul><ul><li>Time seizure activity </li></ul></ul><ul><ul><li>Reorientate </li></ul></ul>Taking control - First Aid Principles
  28. 29. Taking control - First Aid Principles <ul><li>Tonic Clonic Seizures </li></ul><ul><ul><li>Protect from injury </li></ul></ul><ul><ul><li>DO NOT restrain or put anything in mouth </li></ul></ul><ul><ul><li>Time seizure activity </li></ul></ul><ul><ul><li>Recovery position </li></ul></ul><ul><ul><li>Summon help </li></ul></ul>
  29. 30. Taking control - First Aid Principles <ul><li>Tonic Clonic Seizure in Wheelchair </li></ul><ul><ul><li>Brakes & seatbelt on </li></ul></ul><ul><ul><li>DO NOT tilt chair </li></ul></ul><ul><ul><li>If able, manage in wheelchair until jerking stops </li></ul></ul><ul><ul><li>If food, fluid or vomit in mouth, place on side immediately </li></ul></ul>
  30. 31. Taking control - First Aid Principles <ul><li>Tonic Clonic Seizure in Water </li></ul><ul><ul><li>Pool - support head and remove person after jerking stop </li></ul></ul><ul><ul><li>Surf - remove immediately </li></ul></ul><ul><li>Assess ABC, place in recovery position </li></ul><ul><li>Always call ambulance </li></ul>
  31. 32. <ul><li>Call an ambulance if </li></ul><ul><ul><li>Doubt </li></ul></ul><ul><ul><li>Injury </li></ul></ul><ul><ul><li>First seizure </li></ul></ul><ul><ul><li>Tonic clonic longer than 5 min </li></ul></ul><ul><ul><li>Complex partial longer than 15min </li></ul></ul><ul><ul><li>Another seizure quickly follows </li></ul></ul>Taking control - First Aid Principles
  32. 33. <ul><li>Purpose </li></ul><ul><ul><li>Assess risks </li></ul></ul><ul><ul><li>Documented plan of action </li></ul></ul><ul><ul><li>Easy identification of seizure activity </li></ul></ul><ul><ul><li>Step by step instructions </li></ul></ul>Taking control – Seizure Management Planning
  33. 35. Emergency medications are used for the control of seizures that may occur in clusters, prolonged seizures or status epilepticus Taking control – Emergency Medication Training
  34. 36. Taking control – Emergency Medication Training Indications Medical order Epilepsy awareness Training in administration Organisation require medication policy First Aid Certificate
  35. 37. <ul><li>Impact depends upon </li></ul><ul><ul><li>Severity and frequency of seizures </li></ul></ul><ul><ul><li>Age of onset </li></ul></ul><ul><ul><li>Prior knowledge of epilepsy </li></ul></ul><ul><ul><li>Access to support services </li></ul></ul>Taking control – Peer support
  36. 38. <ul><li>Consumer Consultation findings </li></ul><ul><ul><li>Unmet needs </li></ul></ul><ul><ul><li>Unpredictability of seizures </li></ul></ul><ul><ul><li>Social isolation </li></ul></ul><ul><ul><li>Forced dependence </li></ul></ul><ul><ul><li>Impact on family and siblings </li></ul></ul><ul><ul><li>Medication </li></ul></ul>Taking control – Peer support
  37. 39. <ul><li>Peer support programs </li></ul><ul><ul><li>Family Camps </li></ul></ul><ul><ul><li>Youth Camps </li></ul></ul><ul><ul><li>Memory Workshops </li></ul></ul><ul><ul><li>Newly Diagnosed Groups </li></ul></ul>Taking control – Peer support
  38. 40. This is me having a seizure in the classroom. All these things are happening around me and I’m not registering. I’m off in my own little world Amanda 15yrs 156-6/05
  39. 41. 1300 37 45 37
  40. 42. Advances in Tuberous Sclerosis: From Pathway to Therapy Medical and Family Conference 3 – 4 November 2007 Sydney Children’s Hospital Randwick For more information: www.atss.org.au

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