What is the treatment approach for epilepsy due to Rasmussen syndrome in a hospice comfort care setting?

Treatment of Epilepsy in Rasmussen Syndrome within Hospice Comfort Care

In hospice comfort care for Rasmussen syndrome-related epilepsy, prioritize symptom control over seizure elimination using alternative routes of antiepileptic drug administration (buccal, intranasal, subcutaneous, or rectal) with benzodiazepines as first-line agents, avoiding aggressive interventions that would compromise quality of life. 1

Core Principles of Seizure Management in Hospice

The fundamental approach shifts from curative intent to comfort-focused care:

• Treat seizures only when they cause discomfort or reduce quality of life, not for seizure frequency alone 1
• Epileptic seizures affecting quality of life should be treated even with poor prognosis, but anticonvulsant therapy should not impair quality of life more than the seizures themselves 2
• Alternative administration routes (buccal, intranasal, subcutaneous, or rectal) enable effective individualized therapy during the final days of life 1

Medication Selection and Routes

First-Line Agents for Acute Seizures

For breakthrough seizures without IV access:

• Rectal diazepam should be administered as the primary option 2
• Buccal or intranasal benzodiazepines are preferred alternatives when rectal administration is not feasible 1
• Intramuscular phenobarbital may be considered when benzodiazepines cannot be given rectally due to medical or social reasons 2

Palliative Sedation for Refractory Seizures

When seizures remain intractable and cause severe distress:

• Midazolam is the preferred agent for palliative sedation due to its short half-life and rapid onset 2
• Alternative sedatives include phenobarbital, chlorpromazine, or levomepromazine 2
• Doses should be titrated to achieve comfort, not complete seizure suppression 2

Critical Decision-Making Framework

When to Avoid Aggressive Treatment

Aggressive unlimited ICU treatment of refractory status epilepticus in palliative patients is not indicated 1. The following factors support withholding intensive care:

• Metastatic disease involvement, advanced age, high severity of acute illness, overall frailty, and poor functional status before admission all increase probability of poor ICU outcomes 1
• Death in the ICU is often preceded by long and aggressive treatments that may be futile and harmful 1
• Most patients with limited life expectancy would not want aggressive treatment if the anticipated outcome was survival with severe functional impairment 1

Communication and Consent

For terminally ill patients in severe distress where consent cannot be obtained, provision of comfort measures (including palliative sedation if necessary) should be considered standard practice and the default strategy 2

The discussion should include:

• Acknowledgment that prior treatments have not been successful 2
• Current prognosis and rationale for comfort-focused care 2
• Methods available for symptom control, including depth of planned sedation 2
• Reassurance that comfort will be maintained regardless of treatment decisions 2

Practical Implementation

Monitoring and Reassessment

• Capillary blood glucose monitoring can vary from twice daily to once every 3 days depending on patient condition 2
• Continuous reassessment of symptom control is essential, with dose adjustments based on patient comfort rather than seizure frequency 2
• Unnecessary medications, diagnostic tests, and tubes should be removed 2

Family Support

Family members should be informed of the patient's condition, treatment options, and the shift from curative to comfort-focused goals 2. This includes:

• Discussion of the aims, benefits, and risks of palliative approaches 2
• Explanation that HEDIS quality measures do not apply to hospice patients 2
• Education about signs of distress and the plan for managing them 2

Common Pitfalls to Avoid

• Do not pursue aggressive seizure control that compromises comfort or quality of remaining life 2, 1
• Avoid intramuscular diazepam due to erratic absorption 2
• Do not withhold benzodiazepines in actively seizing patients due to concerns about respiratory depression—comfort is the priority 2, 1
• Do not continue disease-modifying immunotherapies (steroids, IVIG) in end-stage hospice care as these do not align with comfort-focused goals 3, 4

Route-Specific Considerations

When vascular access devices for long-term use are already in place, intravenous treatment remains an option even in hospice or home care 1. However:

• Prioritize less invasive routes when possible to minimize discomfort 1
• Subcutaneous administration of medications can be considered as an off-label alternative 2
• Rectal administration remains highly effective and should not be dismissed due to social concerns when patient comfort is at stake 2