Pyridoxine-dependent seizures

Seizure Type	Seizure Sub-Type	Conditions	Triggers/Causes
Pyridoxine-dependent seizures (Other Names: Pyridoxine dependency; Pyridoxine dependency with seizures; Vitamin B6-dependent seizures)	N/A	A condition that involves seizures beginning in infancy or, in some cases, before birth. Those affected typically experience prolonged seizures lasting several minutes (status epilepticus). These seizures involve muscle rigidity, convulsions, and loss of consciousness (tonic-clonic seizures). Anticonvulsant drugs, which are usually given to control seizures, are ineffective in people with pyridoxine-dependent epilepsy. Instead, people with this type of seizure are medically treated with large daily doses of pyridoxine (a type of vitamin B6 found in food).

Those affected by pyridoxine-dependent epilepsy typically experience prolonged seizures lasting several minutes (status epilepticus). These seizures involve muscle rigidity, convulsions, and loss of consciousness (tonic-clonic | Mutations in the ALDH7A1 gene cause pyridoxine-dependent epilepsy. This condition is inherited in an autosomal recessive pattern, which means both copies of the gene in each cell have mutations. The parents of an individual with an autosomal recessive condition each carry one copy of the mutated gene, but they typically do not show signs and symptoms of the condition.

21 J. Osborne et al.,

Evidence of seizure frequency depending on age of patient

There is very little research indicating seizure frequency depending on the age of the patient. Several open source information websites, such as the Australian Medical Association and Consumer Reports reference a substantial 2010 study which suggested that 7 in 10 children diagnosed with epilepsy will grow out of it by the time they are 20. Since that study there appears to be no further substantial research into the subject. None of the open source type literature such as national or state seizure support organisations made mention of this subject, and no other academic research could be sourced.

The above longitudinal study was undertaken by researchers at several hospitals in the Netherlands. The study also concluded that patients can grow out of seizures if they “respond well to early treatment and have fewer seizures in the early years, and if there are no external reason for their epilepsy such as injury or illness”.

The study involved 494 children, each of whom had had at least two seizures starting from when they were aged five on average. “The children were followed up for an average of 15 years, so that the final questionnaire about their symptoms was sent out when the subjects were about 20.

— Genetic and Rare Diseases Information Centre, “Pyridoxine-dependent epilepsy”, [website], 2019, https://rarediseases.info.nih.gov/diseases/9298/pyridoxine-dependent-epilepsy (accessed 9 November 2019) — A. Geerts et al., “Course and outcome of childhood epilepsy: A 15-year follow-up of the Dutch Study of Epilepsy in Childhood”, Epilepsia, Vol 51, No 7, pp. 1189-1197, 2010, https://onlinelibrary.wiley.com/doi/pdf/10.1111/i.1528-1167.2010.02546.x

Types of seizure monitoring devices

There are several types of seizure monitoring devices used as personal/home monitoring as opposed to clinical application:

Mattresses/mats for beds
Smart watches
Mobile phone applications
Pendants
Bracelet/Anklet
Socks (Babies)
Nappies (Babies)
Oximeter

Mattresses/mats for beds

A mattress or mat, which is placed under the mattress. Sensor technology detects and distinguishes between normal movements from strong muscle jerking such as tonic-clonic seizures. Also acknowledges the presence and absence of micro-movements caused by a person’s breathing and heart beating. Gives notification to a caregiver when the person has the clonic phase of a tonic-clonic seizure, but also when the person leaves the bed.

26 Australian Medical Association, “Epilepsy: 7 out of 10 children grow out of it

Smart watches

Reportedly, monitors movements and instantly alerts family members and caregivers upon the onset of repetitive shaking or abnormal patterns of movement. A smart phone with an application will often accompany the watch.

Mobile phone applications (Apps)

Mobile phone applications have a variety of uses in monitoring seizures, where for example, GPS pendants, monitoring cameras, and baby nappy and sock monitors, will send alerts and data to the mobile device via the application. As well, mobile phone applications are widely used to collect seizure data for seizure management where the patient, carer or health professional inputs the data such as the environment and circumstances in which the seizure occurred, time in hours spent in sleep, medication dosage, frequency of alcohol intake, meal times etc. This information is synced and shared with health professionals to assist in seizure management and diagnosis. Such applications are often referred to as seizure diaries. The diagram below indicates the potential use of applications in epilepsy.

28 L. Ranganathan et al., “Application of mobile phones in epilepsy care”, International Journal of Epilepsy, Vol 2, No 1, pp. 28-37, 2015, https://www.sciencedirect.com/science/article/pii/S2213632015000044

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Pendants

Can detect sudden impact of a fall and alert nominated mobile devices and give GPS tracking information to the receiver. Does not detect movement such as shaking or jerking. Other pendants can be simply used as an SOS alert option where the patient presses a button to send an alert.

Bracelet/Anklet

Some pendants can be used as bracelets. A bracelet or anklet device can be worn in conjunction with other monitoring devices. For example, the bracelet/anklet will detect movement when sleeping and send alerts and other information to a mobile phone/tablet.

Cameras

Cameras can be used as sleep activity monitors for caregivers and individuals who need to watch for unusual movements at night. During sleep, audio-video information from a remote infrared video camera is sent to a mobile device with an application installed. When an unusual event is detected, it sounds an alarm and records live audio and video from the camera

Socks (Babies)

Sock worn by babies. Tracks heart and oxygen levels and sends real-time data to a smart phone with an application installed.

Nappies (Babies)

Clips on to the baby’s nappy and monitors breathing movement, sending alerts where breathing movement might stop, to a mobile device with an application installed.

Oximeter

A small clip-like device which monitors the oxygen saturation of a patient’s blood (as opposed to measuring oxygen saturation directly through a blood sample). Small beams of light pass through the blood in the finger, measuring the amount of oxygen.

Device suitability depending on age of patient

Apart from monitors which attach to a baby’s nappy and the sock monitor for babies, all other devices appear to be suitable for all age groups. Note: The research would indicate that adults may be more able than children to have knowledge and alert to the onset of some types of seizures.

Seizure Monitoring Products

Below is a selection of seizure monitoring products based on what appears to be the most popular according to prominent community epilepsy organizations.

Product Name	Device Type	Link to Supplier(s)
Embrace 2	Smartwatch	https://www.empatica.com
Enspyre	Smartwatch	https://smart-monitor.com
Apple Watch Series 5	Smartwatch	https://www.apple.com/au/apple-watch-series-5/health
My Medic Watch	Mobile phone/watch App	https://www.mymedicwatch.com

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Evidence of efficacy of seizure monitoring devices

It appears that there is no evidence of efficacy, and no formal selection or prescription guidelines for seizure monitoring devices.
For most of the device types, no reference could be found with regard to efficacy and how a patient may choose a particular device for their particular seizure.

The majority of literature on the devices is manufacturer marketing and general open source type. Prominent epilepsy organisations, such as Epilepsy Action Australia , and the Epilepsy Foundation in the US, only discuss the types of monitoring devices and what seizure types they might assist to monitor, with links to manufacturer sites for purchase.

Epilepsy Action Australia asserts that “there are countless products on the market that can help people with seizures or their families feel more comfortable about safety. Please note that monitors, alarms and safety products don’t guarantee safety or detection of all seizures, but they can provide peace of mind for some people”. The American Epilepsy Foundation suggest that “no alert device has been designed to prevent seizures or possible impact from seizures. There are no devices available that have been proven to prevent sudden unexpected death in epilepsy (SUDEP). Yet, since SUDEP most often occurs during sleep, some people with seizures at night may be helped by having a way to let others know if a seizure occurs”.

No lived experience or opinion regarding referral or prescription from GP’s or specialists could be found. There appears to be little research interest in device monitoring from professionals in the field, however there appears to be a rising trend in academic research of monitoring devices including monitoring at a clinical level.

In 2018 the NDIS Technical Advisory Team conducted a face-to-face survey of three experts in the field of Neurology and Epileptology. (See Appendix A). The survey evolved from the high rate of

2° Epilepsy Action Australia, Safety Products and Alarms, [website], 2019, https://www.epilepsy.org.au/e-360-edition-13-safety-products-and-alarms, (accessed 19 August 2019). 3° Epilepsy Foundation, The Role of Seizure Alerts, [website], 2019, https://www.epilepsy.com/learn/early-death-and-sudep/sudep/role-seizure-alerts, (accessed 19 August 2019).

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Product Name	Device Type	Link to Supplier(s)
SeizAlarm App	Mobile phone/watch App	http://seizalarm.com
SeizureTracker	Mobile phone App	https://www.seizuretracker.com
Keep Track GPS	Pendant	https://www.keeptrackgps.com.au/products/personal-alarm-telstra-network
Life Minder	Pendant	http://lifeminder.com.au/product/the-life-minder
LiveLife	Pendant	https://livelifealarms.com.au
Sami	Camera	https://www.samialert.com
Pulse Guard	Bracelet/Anklet	https://pulseguard.org
Epi Assist	Mattress/Mat	http://www.epiassist.com.au
Owlet	Socks (Babies)	https://owletcare.com.au
Szuna	Nappies	https://www.snuza.com
Edan	Oximeter	https://amamedicalproducts.com.au/collections/pulse-oximeters/products/edan-h100b-hand-held-pulse-oximeter

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participants requesting 2:1 seizure monitoring from support workers. The experts were asked if the need for face to face monitoring might be reduced with the use of monitoring or alarm devices, and what monitoring devices would be appropriate for what types of epilepsy?

Only one expert commented that the devices would need to be sensitive: “This depends upon the type of seizure, the time of day/night and so on. The device needs to be sensitive without too many false alarms. Again, this has to be worked out on an individual basis”. Another expert commented with regard to the 2:1 support worker support: ‘These would be exceptional cases in which there is a severe seizure disorder with severe underlying neurological disability. Superficially, it would seem reasonable for 1 person if prompt ambulance assistance was available. As discussed, for a person to need RN level care or 2:1 care for (potential) seizures, they would have to have a high-level of additional medical problems/equipment that lead to such severe compromise or need for intervention.’

A recent significant literature review research paper on seizure monitoring devices concluded that ‘there are limited data on which is the best sensor for each seizure type. It is likely that multimodal patient-specific detection systems will be needed to meet the complex requirements of seizure detection’.

The table below is an overview of some of the devices. Evidence of efficacy and in what case a device might be chosen, could not be found for most device types.

Evidence of Efficacy

Device Type	Description	Primary Input	Evidence of Efficacy	In what case would device be chosen?
Seizure mats/mattresses for beds	Will monitor a sleeping adult or child’s convulsive seizures from under the mattress.	Movement	Allows nocturnal generalized tonic-clonic seizures detection. There is no need to place electrodes on the patient. Parameters can be adjusted for every patient. Only detects seizures with rhythmic movements. There is a weight limitation. Some find it uncomfortable. Low sensitivity. (Key H from Table B). 32	Generalized tonic-clonic seizures, Focal dyscognitive seizures with motor phenomenon. (Key H from Table B)
Smart watches	Several types of Smart Watches with similar functions: helps detect seizures and monitor activity and sleep. Linked to the wearer’s	Movement, Motion, GPS, electrical activity of skin

31 A. Ulate-Campos et al.

Device Type	Description	Primary Input	Evidence of efficacy
Pendants	When the user needs help they press the button, and the pendant will text and call emergency contacts. Each text message will show their location on Google Maps using the built-in GPS. When the call from the pendant is answered they can speak and listen ‘hands free’ through their pendant. 34	User, GPS
Harness/Drop Harness	Upper body harness that wraps around the user’s waist, chest and torso, with an attached central handle running the length of the torso. 35	Movement, Motion
Socks (Babies)	Smart Sock fits snug on baby’s foot and monitors their heart rate and oxygen levels while they sleep. 36	Movement, heat rate
Nappies (Babies)	Wearable device which attaches to baby’s nappy and monitors abdominal movement. 37	Movement, motion
Oximeters	Measures oxygen saturation level, or the oxygen levels in blood. It can rapidly detect even small changes in how efficiently oxygen	Pulse	To date the reliability of these devices has not been robustly assessed in a patient population. *9 The Thoracic Society of Australia and New

Choosing a device type: guidance for physicians, families and researchers

A recent significant literature review research paper summarized current evidence, and offered suggestions on how to select the most suitable seizure detection device for each patient and provide guidance to physicians, families and researchers when choosing or designing seizure detection devices. However, it did conclude that there is limited data on which is the best sensor for each seizure type. The table below is a comparison of the available seizure prediction sensors.

Note that some of these sensors are for clinical use.

KEY	Seizure detector	Primary input	Seizures detected	Advantages	Disadvantages
A	Electroencephalogram (EEG)	EPSP/IPSPs	Focal dyscognitive seizures, focal without dyscognitive changes and secondarily generalized, absence seizures	Gold-standard. Good sensitivities were achieved	Discomfort, stigmatization due to electrodes. Risk for movement artefact

38 Epilepsy Action Australia, Fact Sheet: Seizure Safety Products, [website], 2019, hhttps://www.epilepsy.org.au/wp-content/uploads/2019/07/Fact-Sheet-Seizure-Safety-Products.pdf, (accessed 19 August 2019). 40 J. Pretto, Clinical use of pulse oximetry: Official guidelines from the Thoracic Society of Australia and New Zealand, 2013 (https://www.thoracic.org.au/clinical-documents/command/download_file/id/34/filename/Pretto_et_al-2014-Respirology.pdf) 41 Epilepsy Action Australia, Seizure Monitors & Wearable Technology [website], 2017, hhttps://www.epilepsy.org.au/how-we-can-help/epilepsy-products, (accessed 16 August 2019). 42 Epilepsy Action Australia, Fact Sheet: Seizure Safety Products, [website], 2019, hhttps://www.epilepsy.org.au/wp-content/uploads/2019/07/Fact-Sheet-Seizure-Safety-Products.pdf, (accessed 19 August 2019). 43 A. Ulate-Campos et al.

KEY	Seizure detector	Primary input	Seizures detected	Advantages	Disadvantages
B	Intracranial EEG	EPSP/IPSPs	Focal dyscognitive seizures, focal without dyscognitive changes and secondarily generalized	Gold-standard. Good sensitivities, were achieved	Invasive procedure with potentially life-threatening complications
C	Surface electromyography (sEMG)	Muscle movement	GTCS, tonic seizures, hypermotor seizures	Early detection in tonic phase with one channel. Good sensitivity and nighttime detection	Only detects seizures with a motor component. Electrodes could detach, move or be uncomfortable
D	Electrodermal activity (EDA)	Sweating	GTCS, Focal dyscognitive seizures	Good detection rates, including FDS, better when combined with ACM	Susceptible to motion and pressure artifacts, could be uncomfortable
E	Electrocardiography (EKG)	Heart rate changes	Focal seizures, secondarily generalized seizures and GTCS	Heart rate is relatively easy to detect. Can be recorded from one channel. Higher signal-to-noise ratio than EEG	Seizures without HR changes go undetected. HR changes occur in many everyday activities and seem to be patient-specific. Electrodes might be uncomfortable or unstable on the long-term
F	Accelerometry (ACM)	Movement	GTCS, secondarily generalized, myoclonic, clonic, tonic and hypermotor seizures	Great sensibility, good night detection rates. User-friendly	Only detects seizures with a motor component and when there is free limb movement. Battery system needs to be optimized
G	Video detection systems	Movement	Focal, hypermotor, myoclonic and clonic	No discomfort for patients in marker-free video	Mainly detects seizures with a motor component. Limited to the area covered by video, the patient must be visible and properly placed. Attached markers could dislocate or produce discomfort
H	Mattress sensors	Movement, noise	GTCS, Focal dyscognitive seizures with motor phenomenon	Allows nocturnal GTCS detection. There is no need to place electrodes on the patient. Parameters can be adjusted for every patient.	Only detects seizures with rhythmic movements. There is a weight limitation. Some find it uncomfortable. Low sensitivity
I	Seizure-alert dogs	Subtle behavioral changes are detected by the dogs	Focal dyscognitive seizures, GTCS	Alert before the seizure. Increase in QOL, possible reduction in seizure frequency	Few studies while the patient is on EEG. They also alarm to psychogenic seizures. Cannot detect while dogs sleep
J	Cerebral oxygen saturation sensors	Cerebral blood flow	GTCS, temporal lobe seizures	Can detect seizures up to 18min before clinical onset	Few studies, only in 2 seizure types

Future Research

A 2015 comprehensive research review explored possibilities in the field of epileptology after analysing the current and existing applications of mobile phones in care of the epileptic patients worldwide. The review suggested that “mobile phone apps can be useful in the hands of the epileptic patients, their caregivers or the healthcare professionals themselves”. It goes on to suggest that “A multi-disciplinary approach involving the collaboration of neurologists, electronic and electrical engineers, pharmaceutical companies and more importantly the patients themselves would be required to make such possibilities a reality”.

From the research sourced little could be found regarding testing and trials of the monitoring devices. It is very clear that further research is required into the efficacy of seizure monitoring devices.

A 2017 research article looked at standards for testing and clinical validation of seizure detection devices, and suggested that

Research Summary

There are several types of seizures some of which are classified as epileptic.
There is a broad range of causes and triggers which may apply to all seizure types. In some seizure types causes or triggers are unknown.
A seizure alert device is a monitoring system that can detect when a person may be having a seizure and notify someone who can respond.
There are several types of seizure monitoring devices using different type of sensory and input processes.
All devices researched appear to be suitable for use by all age groups, except baby specific items.
There is no solid evidence that seizure frequency increases or decreases depending on the age of the patient, and research in this area is limited.
It appears that there is no evidence of efficacy of the devices. No research trials or tests could be sourced, and there is a running theme in the literature for the need for further research.

Very little reference could be found with regard to how a patient may choose a particular device for a particular seizure type.

s47E(d) - certain operations of agencies

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Reference List

A. Geerts et al., “Course and outcome of childhood epilepsy: A 15-year follow-up of the Dutch Study of Epilepsy in Childhood”, Epilepsia, Vol 51, No 7, pp. 1189-1197, 2010, https://onlinelibrary.wiley.com/doi/pdf/10.1111/j.1528-1167.2010.02546.x
A. Ulate-Campos et al., “Automated seizure detection systems and their effectiveness for each type of seizure”, Seizure, Volume 40, August 2016, Pages 88-101. https://www.sciencedirect.com/science/article/pii/S1059131116300711
A. Ulate-Campos et al., “Automated seizure detection systems and their effectiveness for each type of seizure”, Seizure, Volume 40, August 2016, Pages 88-101. https://www.sciencedirect.com/science/article/pii/S1059131116300711
Australian Medical Association, “Epilepsy: 7 out of 10 children grow out of it”, [website], 2019, https://ama.com.au/ausmed/epilepsy-7-out-10-children-grow-out-it, (accessed 9 December 2019)
Cedars Sinai, “Atonic Seizures”, [website], 2019, https://www.cedars-sinai.edu/Patients/Health-Conditions/Atonic-Seizures.aspx?_ga=2.105746691.1656684614.1575939601-329177124.1575939601, (accessed 9 December 2019)
Cedars Sinai, “Simple Partial Seizures”, [website], 2019, https://www.cedars-sinai.edu/Patients/Health-Conditions/Simple-Partial-Seizures.aspx?_ga=2.63216303.1656684614.1575939601-329177124.1575939601, (accessed 9 December 2019)
Epilepsy Action Australia, “Psychogenic non-epileptic seizures”, [website], 2017. https://www.epilepsy.org.au/epilepsy-trainer-news-feature-psychogenic-non-epileptic-seizures, (accessed 26 November 2019)
Epilepsy Action Australia, “Safety Products and Alarms”, [website], 2019. https://www.epilepsy.org.au/e-360-edition-13-safety-products-and-alarms, (accessed 19 August 2019)
Epilepsy Action Australia, “Seizure Types”, [website], 2019. https://www.epilepsy.org.au/about-epilepsy/understanding-epilepsy/seizure-types-and-classification, (accessed 25 November 2019)
Epilepsy Foundation of America, “Considering a seizure alert device”, 2018 [online brochure]. [https://www.epilepsy.com/sites/core/files/atoms/files/DAS100 Seizure Alert Devices 09-2018 FINAL2.pdf](https://www.epilepsy.com/sites/core/files/atoms/files/DAS100 Seizure Alert Devices 09-2018 FINAL2.pdf), (accessed 19 August 2019)
Epilepsy Foundation, “Focal Onset Impaired Awareness Seizures (complex partial seizures)”, [website], 2019. https://www.epilepsy.com/learn/types-seizures/focal-onset-impaired-awareness-seizures-aka-complex-partial-seizures, (accessed 9 December 2019)
Epilepsy Foundation, “Infantile Spasms (West’s Syndrome) and Tuberous Sclerosis Complex”, [website], 2019. https://www.epilepsy.com/learn/types-epilepsy-syndromes/infantile-spasms-wests-syndrome-and-tuberous-sclerosis-complex, (accessed 9 December 2019)

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Epilepsy Foundation, “Seizure-Alert Dogs: Just the Facts, Hold the Media Hype”, [website], 2019, https://www.epilepsy.com/article/2014/3/seizure-alert-dogs-just-facts-hold-media-hype, (accessed 20 January 2020)

Epilepsy Foundation, “Tonic Seizures”, [website], 2019, https://www.epilepsy.com/learn/types- seizures/tonic-seizures, (accessed 9 December 2019)

Epilepsy Foundation, “Will I Always Have Seizures?”, [website], 2019, https://www.epilepsy.com/learn/about-epilepsy-basics/will-i-always-have-seizures, (accessed 11 December 2019)

Epilepsy Foundation, The Role of Seizure Alerts, [website], 2019, https://www.epilepsy.com/learn/early-death-and-sudep/sudep/role-seizure-alerts, (accessed 19 August 2019).

Genetic and Rare Diseases Information Centre, “Pyridoxine-dependent epilepsy”, [website], 2019, https://rarediseases.info.nih.gov/diseases/9298/pyridoxine-dependent-epilepsy, (accessed 9 November 2019)

J. Osborne et al., “Infantile Spasms and West Syndrome: An explanatory booklet for parents and for professionals”, 2006, https://www.rch.org.au/uploadedFiles/Main/Content/neurology/20060215 InfantileSpasms han dout.pdf

John Hopkins Medicine, “Absence Seizures”, [website], 2019, https://www.hopkinsmedicine.org/health/conditions-and-diseases/epilepsy/absence-seizures, (accessed 9 December 2019)

John Hopkins Medicine, “Tonic and Clonic Seizures”, [website], 2019, https://www.hopkinsmedicine.org/health/conditions-and-diseases/epilepsy/tonic-and-clonic- seizures, (accessed 9 December 2019)

L. Ranganathan et al., “Application of mobile phones in epilepsy care”, International Journal of Epilepsy, Vol 2, No 1, pp. 28-37, 2015, https://www.sciencedirect.com/science/article/pii/S2213632015000044

Mayo Clinic, “Grand mal seizure”, [website], 2019, https://www.mayoclinic.org/diseases- conditions/grand-mal-seizure/symptoms-causes/syc-20363458, (accessed 28 November 2019) Mayo Clinic, “Seizures”, [website], 2019, https://www.mayoclinic.org/diseases- conditions/seizure/symptoms-causes/syc-20365711, (accessed 9 December 2019)

National Institute of Neurological Disorders and Stroke, “Febrile Seizures Fact Sheet”, [website], 2019, https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Febrile- Seizures-Fact-Sheet, (accessed 28 November 2019)

National Institute of Neurological Disorders and Stroke, “Grand mal seizure”, [website], 2019, https://www.ninds.nih.gov/Disorders/Patient-Caregiver-Education/Fact-Sheets/Myoclonus-Fact- Sheet, (accessed 28 November 2019)

Queensland Paediatric Epilepsy Network, New Diagnosis of Epilepsy Fact Sheet“, https://www.childrens.health.qld.gov.au/wp-content/uploads/PDF/qpen-new-diagnosis- booklet.pdf

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Appendix A: NDIS Technical Advisory Team Survey

2018 NDIS Technical Advisory Team Survey of three experts in the field of Neurology and Epileptology.

Survey Responses

NOTE: Professor Mark Cook did not return responses.

Questions

Is face to face continuous monitoring of a person who experiences seizures ever required or recommended for a person (child or adult) with epilepsy only and no other comorbidities? (ie continuous watching of the person in case a seizure occurs).

Harvey	Yes (but rare)
Newton	This would be a very rare situation. Most people with such severe epilepsy usually have co-morbidities.
Cook

If yes, in what circumstances would this be required? Please comment on different Epilepsy types and level of risk of seizures for different persons with Epilepsy (other than intractable epilepsy). Please provide details of the following:

Type of observation and actions required
Record keeping recommendations/ requirements
Mandatory qualifications and experience of support workers

Research Request - Epilepsy & Seizure Monitoring Systems

Harvey

Typically infants with severe seizures disorders (eg. multiple hourly seizures with depressed airway/breathing/circulation during the seizures) who may have emerging developmental delays but not an established diagnosis of a comorbid disability. This is often provided by parents and carers who have been trained by medical and nursing staff. This may involve recording of seizure frequency/duration, giving regular medication, providing airway management +/- O2 if used at home, administration of midazolam, calling for ambulance support when necessary.

Newton

The situation here is likely determined by Lennox Gastaut Syndrome (LGS) where tonic seizures or atonic seizures (drop attacks) can be frequent and potentially injurious. Usually a parent or carer keeps close by while patient is on their feet. Recording the events is necessary as guide for medical review. Training in seizure care is usually provided by the Epilepsy support groups, such as Epilepsy Australia and Epilepsy Foundation Victoria.

Cook

Is face to face continuous monitoring of a person experiencing seizures ever required for a person (child or adult) with epilepsy where there are co-morbidities? (eg Intellectual disability, ASD, CP)

Harvey

Yes, more commonly, as the underlying neurological problems causing such severe epilepsies tend to also cause other neurological comorbidities that may exacerbate the effects of seizures eg. tonic or tonic-clonic seizures in a child with CP.

Typically these would be children or adults with ID, CP, airway/feeding issues (eg. Rett syndrome, severe brain injuries, severe brain malformations) who have a high level of baseline disability and need for care who become additionally compromised by their seizures. These people would likely have parents/carers who need to respond to seizures with positioning, suctioning, maybe O2 and emergency medication. Monitoring would typically be assisted by oximetry, so that they don’t need face-to-face.

Newton

Although uncommon, there are people with frequent and potentially risky seizures who need watching.

Cook

If yes, in what circumstances would this be required? Please comment on different Epilepsy types and level of risk of seizures for different persons with Epilepsy (other than intractable epilepsy). Please provide details of the following:

Type of observation and actions required
Record keeping recommendations/ requirements
Mandatory qualifications and experience of support workers.

Harvey

Again, this would typically be in a child, adolescent or adult with severe epilepsy (such as Lennox-Gastaut syndrome resulting from a genetic disorder,

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Newton	The circumstances are usually epilepsy with co-morbidity such as developmental or acquired brain disorders. Action may amount to positioning of the person during and after seizure, to airway management, emergency medication administration such as midazolam via buccal or nasal routes. Seizures are usually charted noting if medication is given, to allow better appraisal at next neurology clinic review. Basic seizure first aid and when necessary training in emergency medication administration is required. This is usually provided by Epilepsy support groups, with the help of guidelines provided by the treating neurologist.

Cook

Is face to face continuous monitoring of a person experiencing seizures ever required for a person (child or adult) with intractable epilepsy both with and without co-morbidities? (eg Intellectual disability, ASD, CP)

| Harvey | Potentially, in some instances. The seizures need to be of a type that the specific monitor can detect with extremely high reliability eg. movement detectors for TCS, apnoea monitor for seizures with apnoea. For the children/adults described above, oximetry would typically be used because they have limited mobility (oximeter won’t fall off) and seizures compromise their breathing. Oximeters typically need to be rotated between digits, by the carer or parent. | | Newton | See (4) above. | | Cook |

If yes, in what circumstances would this be required? Again, please comment on :

Type of observation and actions required
Record keeping recommendations/ requirements
Mandatory qualifications and experience of support workers

| Harvey | This is a huge topic for which I have no particular expertise. Parents/carers typically research and try out different devices to find one that is suitable for their child. However, these are often for people with mild epilepsy having infrequent seizures to provide peace of mind, especially at night. For more expert opinion, I would suggest contacting Epilepsy Australia/Action or the team at St Vincent’s Hospital Melbourne who work in this space. As discussed, the extremely high care patients with parents or in-house 1-on-1 carers would probably be using oximeters or apnoea mattresses. Other |

ndis

| devices need to be shown to be effective at detecting seizures and having low false alarm rates. The monitoring for infrequent seizures (to prevent SUDEP) in people with or without disability is a separate issue. In the absence of other disability, this is probably not an NDIS issue. I spoke with Mark Cook at SVHM who would be happy to speak with you. | Newton | (Left blank) | Cook

What specific support is required at the time a person (adult or child) is having a seizure or in the post ictal period??

Harvey I am not aware of any high-quality medical evidence for the systematic use of “seizure alert dogs”. However, I can imagine that there are individual cases in which a particular dog alerts carers to a particular person’s specific seizures. s47F - personal privacy

Newton This depends on the particular case in question. Some seizures have little recovery periods, others are much longer and require more oversight. If confused, there needs to be observation, reassurance if needed, and physical intervention, such as preventing a person from wandering in the confused state. Eg. If an exit door cannot be locked, a carer may stand in front of the door. Actual handling is best avoided in most cases. Should there have been an injury in the seizure, carer would report to supervisor on the nature of it to ascertain whether medical review is necessary.

Cook

Can this support be provided by a trained support worker or parent? If not, what qualifications would be required in any circumstances?

Harvey See first aid advice on any of the epilepsy support group websites — positioning, monitoring, protecting, calling for emergency assistance if prolonged, administering midazolam if prescribed and trained, postictal care.

Newton Yes, this is usually done by trained support worker or parent.

Cook

Can this support be provided by a trained support worker or parent? If not, what qualifications would be required in any circumstances?

Harvey Yes.

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ndis

The agency has also been receiving requests to provide up to 2 support workers to accompany participants either in the home or out in community so there is support available to assist at all times in case of an emergency including administration of Midazolam, and resuscitation with respiratory bagging, administration of oxygen and trachea maintenance.

Would 2 people ever be required to assist with management of a seizure when there is access to on call ambulance services? If so, in what circumstances?
What qualifications, if any, are required of the second person (if required)? Is there any reason a second person would be required in case of an emergency where there is access to ambulance services?

| Harvey | These would be exceptional cases in which there is a severe seizure disorder with severe underlying neurological disability. Superficially, it would seem reasonable for 1 person if prompt ambulance assistance was available. As discussed, for a person to need RN level care or 2:1 care for (potential) seizures, they would have to have a high-level of additional medical problems/equipment that lead to such severe compromise or need for intervention. This might be a line in the sand that contrasts NDIS and Health. | | Newton | There are exceptional cases where due to patient’s physical size, degree of immobility, duration and type of seizure when 2 people will better manage a seizure than one. It is very much dependent on the individual case. In my adult clientele, there are only 2 or 3 cases who come into this category. | | Cook | |

Can the need for face to face monitoring be negated or reduced with the use of monitoring or alarm devices?

Newton This depends upon the type of seizure, the time of day/night and so on. The device needs to be sensitive without too many false alarms. Again, this has to be worked out on an individual basis. Cook |

If so, what monitoring devices would be appropriate for what types of epilepsy? Please comment on the evidence base for any monitoring/ alarm devices.

Newton Please see Simon Harvey & Mark Cook responses. Cook |

Is there any evidence for the use of seizure alert dogs for the monitoring and management of epilepsy? Newton My review of the literature does not reveal a strong case at this point in time for seizure alert dogs.

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| Cook

Details of experts surveyed

Consultant	Detail	Contact
Dr. Mark Newton	Neurologist & Epileptologist; Epilepsy specialist with experience with severe epilepsy and associated co-morbidities; senior staff from the Epilepsy Research Centre	Epilepsy Research Centre
Profile: Link

Epilepsy Research Centre Level 2, Melbourne Brain Centre 245 Burgundy Street Austin Health Heidelberg 3084 VIC AUSTRALIA Telephone: +613 9035 7330 Email: redacted@unimelb.edu.au

Melbourne Uni: Profile: Link | | Dr Simon Harvey | Consultant Neurologist; Director, Children’s Epilepsy Program | RCH Link

Phone: (03) 9345/9“) Department of Neurology The Royal Children’s Hospital 50 Flemington Road Parkville, Victoria 3052 Australia the RCH Neurology Department, with additional appointments in the MCRI and University of Melbourne Department of Paediatrics. Profile: Link | | Professor Mark Cook | Currently Chair of Medicine at St. Vincent’s Hospital; Professor Cook specialises in the treatment of epilepsy — his previous role was at St Vincent’s as Professor and Director of Neurology. He is recognised internationally for his expertise in epilepsy management, particularly imaging and surgical planning. | St Vincent’s Level 5, Daly Wing, St Vincent’s Hospital 41 Victoria Parade, Fitzroy VIC 3065 Phone: (03) 9231/73 Email: redacted Profile: Link

Melbourne Uni: Link |

Appendix B: NDIS Health Policy (Epilepsy monitoring through assistive technology)

Epilepsy monitoring through assistive technology	NDIS Responsibility
Epilepsy assistive technology is to monitor seizures which directly improves the functional capacity of the participant and their abilities to undertake activities of daily living.	The NDIS will fund this disability related health support when it is a regular part of the participant’s daily life and results from the participant’s disability.
Epilepsy monitoring through assistive technology (seizure monitor alarm systems and seizure mats for beds).
In this instance, the rationale for providing the assistive technology for epilepsy monitoring is because it may reduce or negate the need for a support worker to provide face to face monitoring.

Health System Responsibility

All supports provided in a hospital setting or when not a regular part of the participant’s daily life or resulting from the participant’s disability