ICE Epilepsy Alliance is a member of the American Epilepsy Society’s Vision 20/20 Task Force, a group of advocacy groups committed to improve the lives of patients with epilepsy.
At the request of the Department of Health and Human Services Office of the Assistant Secretary for Planning and Evaluation, the National Institutes of Health, and Vision 20/20 the Institute of Medicine will convene an ad hoc committee to recommend priorities in public health, healthcare and human services, and health literacy and public awareness for the epilepsies and to propose strategies to address these priorities. The committee will focus its work on the following four topic areas:
1) Public Health Surveillance, Collection, and Data Integration: Examine how existing or new surveillance systems could support a more accurate assessment of the public health burden of the epilepsies for patients and their families;
2) Population and Public Health Research: Identify what research questions or areas of focus should be priorities for future epidemiological and population health studies on the epilepsies that may inform the development of interventions or preventive strategies;
3) Health Policy, Healthcare and Human Services: Identify what constitutes adequate care and access to health and human services for people with epilepsy; what can be done to improve the consistency and quality of care for persons with epilepsy; what gaps and needs for improvement exist. Discussion is needed on maximizing community inclusion and personal outcomes for persons with epilepsies (e.g., changes in public health and health services policies and practices or community and family-based support programs).
4) Patient, Provider, and Public Education: Define what needs exist to improve the education and training of health and other professionals who treat or support persons with epilepsy. Additionally, explore how public education and awareness campaigns could best be used to increase patient and public literacy, reduce stigma, and improve community support and participation for people with epilepsy.
ICE Epilepsy Alliance has chose #3, Healthcare Reform, as a platform to be presented to the IOM at the first public panel in January:
The needs of children with epileptic encephalopathies are complex and require a multi-disciplinary medical team. The International League Against Epilepsy (ILAE) describes “epileptic encephalopathy” as the notion that the epileptic activity itself may contribute to severe cognitive and behavioral impairments above and beyond what might be expected from the underlying pathology alone, and that these can worsen over time.1 Inherent to this concept is the idea that by suppressing or preventing the epileptic activity, one may improve the cognitive and behavioral outlook of the disorder. In the developing brain, this concept has lead to the hope that rapid effective intervention should and can be used before the abnormal epileptic activity interferes irrevocably with normal processes of brain development. Fortunately, only 2.5-4.1% of newly diagnosed cases of childhood epilepsy are diagnosed with an epileptic encephalopathy.2
Most children with epileptic encephalopathies develop multiple disabilities including developmental delays and are chronically medically unstable, putting them at high risk for frequent seizure emergencies such as prolonged ( > 5 minutes) or repetitive seizures (> 2 in 24 hours) and status epilepticus (> 30 minutes). These children have frequent emergency room and hospital visits and are at a 24x increased risk of sudden death compared to their peers. This devastating phenomena, known as Sudden Unexplained Death of Epilepsy (SUDEP), along with increased risk of death due to seizure or accidents such as drowning is the day-to-day (or minute-to-minute) reality these children face. Status epilepticus is associated with morbidities such as brain damage and further developmental delays and will result in death if not resolved. Prevention of status epilepticus is essential for the safety and developmental outcome of these children. Additionally, many of these children have drug resistant epilepsy, which necessitates trial and error of multiple drug regimens leading to a complex and ever changing plan of care.
For optimal care, these children require 24/7 monitoring by a caregiver who can:
- Recognize and respond to a seizure emergency onset and cessation (there is a high incidence of non-convulsive status epilepticus in these children – no visible signs of seizure activity but brain is still seizing)
- Administer often high doses of drugs known as benzodiazepines to abort or prevent a seizure emergency, which may compromise breathing
- Monitor breathing and heart rate and administer oxygen
- Clinically asses the unstable seizing child in acute situations and on an ongoing basis to provide feedback to the neurologist to optimize the plan of care
- Understand the signs and symptoms of possible drug toxicity of the multiple drug regimen these children require, as most children are on three or more anti-epileptic drugs three or more times per day
- Coordinate the frequent changes in the plan of care
It is a struggle for parents to dedicate 24/7 to their child with an epileptic encephalopathy, however this level of monitoring is necessary for the safety of the child. While parents of these medically fragile children become quickly attuned to the child and their needs, most parents are not medically trained and live in a constant state of fear and anxiety that the next seizure will be the one that takes the child away or that it will leave the child completely neurologically impaired. Parents are unable to find competent caregivers for the child due to the complex medical needs.
Skilled nursing care for children diagnosed with epileptic encephalopathies who have a history of frequent seizure emergencies is necessary for the safety and best clinical outcome of the child. Skilled nurses provide emergency medical assistance, co-ordination of care and follow through with the child neurologist on the frequent anti-epileptic drug changes, and clinical assessment of the child during and in between seizure emergencies and changes in plan of care.
There is an unmet need and gap in coordination of the care of these children between state Medicaid, private health insurers, and the public school system. Medicaid policies for medically fragile children vary from state to state and often parents are not aware that skilled nursing care is an option or that the child may be eligible for Medicaid regardless of the parent’s income. Private insurance policy provision of skilled nursing is defined by the individual policy and parents often are unaware that this is an option for their child. Medicaid and private insurance administrators do not understand the skilled nursing needs of the medically unstable child with an epileptic encephalopathy. While these children may meet the criteria of a “medically unstable patient with complex medical needs that requires skilled nursing to facilitate the plan of care”, the insurer often rejects a skilled nursing request and dismisses the need for nursing for a child who simply has “epilepsy” or states that what is being requested is “custodial care” that does not meet the policy requirements for skilled nursing. Administrators typically grant skilled nursing to children who are in a “trach and vent” situation in which skilled nursing is required to respond to the bells and whistles on a machine that alert to the need for a nursing intervention. Unfortunately, children with epileptic encephalopathies having frequent and life threatening seizure emergencies are not equipped with bells and whistles to alert someone to intervene, and we argue that due to the unpredictable nature of this disease, greater clinical aptitude in the skilled nurse is required for the care of these children compared to the “typical” child who requires skilled nursing care. We ask that insurers look at the big picture of the burden of illness in this rare group of children, including the overall costs associated with suboptimal medical management and intervention by skilled nurses not limited to frequent ambulance, emergency room, hospital, and intensive care visits but extending to the co-morbidities associated with frequent seizure emergencies and the resulting long term costs and compromised quality of life.
Section 504 of the Rehabilitation Act of 1973 dictates that the public school system is obligated to provide a Free and Appropriate Education (FAPE) to students with disabilities. Most parents are not told that children in which developmental delays are anticipated based on their diagnosis, regardless of age, are eligible for an Individualized Education Plan (IEP) and services through the public school system. These serviced include therapies and attendance of pre-school if the child is able, or provision of an itinerate teacher and home based therapy if the child is not able to attend school. If the child neurologist determines it is unsafe for the child to attend school without a 1:1 skilled nurse to assist, the public school system must provide this service. Enriched environment through interaction with typical and non-typical peers is critical to optimizing developmental, social, and emotional outcome of these children.
ICE Epilepsy Alliance encourages the epilepsy community to help address this unmet need in skilled nursing care for children with epileptic encephalopathies who are medical unstable and have a history of frequent prolonged seizures. We must educate administrators of healthcare policy reform, administrators of private insurance policies and Medicaid, and the public school system about the intense medical needs of this small subset of children in which the burden of their medical needs is often dismissed as a “a seizure disorder” and not understood to be the serious and often fatal disease these children face.
2. Thambyayah M. Early epileptic encephalopathies including West syndrome: A 3 -year Retrospective Study from Klang Hospital, Malaysia. Brain Dev 2001;23 (7): 603-04.