To all ICE advocates: This letter was written by a parent of a child with Dravet syndrome who appealed to the Ohio state Medicaid department for nursing services to care for her child. Each state has a different policy and plan for developmentally disabled children and their care, dependent on level of need. Please look for additional articles and letters on pursuing care for children with ICE, and where to look.
Waiver name: Level One
Description: This waiver allows MR/DD persons of all ages who meet the ICF/MR level of care to remain living at home and in the community. Services offered under this waiver include: daily living services, nursing services, home delivered meals, and adaptive/assistive devices. Waiver is currently used for people who are in the Ohio Home Care Waiver, but who have an ICF/MR level of care.
Level One contact information
State waiver website:
Level One website
Level One 2006 participant data
Level One 2006 expenditure data
October 1, 2010
Dear Emergency Review Committee,
Our son, JS, has the unfortunate clinical and genetic diagnosis of a malignant and catastrophic epileptic syndrome known as Severe Myoclonic Epilepsy of Infancy (Dravet syndrome). We have been educating ourselves about his newly defined diagnosis for the past several months. Dravet Syndrome is a rare genetic syndrome due to a gene mutation that interferes with protein development in sodium channels in the brain. The faulty sodium channel causes febrile and non-febrile seizures (mostly life-threatening status epilepticus) in previously healthy infants. The incidence of Dravet syndrome is 1:20,000 – 1:40,000, however, less than 1500 have been diagnosed in the United States with a probable 16,000 cases existing. The genetic test became commercially available in the United States six years ago, and doctors are learning more about the scope and spectrum of this epileptic syndrome.
Dravet Syndrome is characterized by a constellation of seizures presenting during the first year of life. It is progressive, debilitating, and often includes frequent life-threatening status epilepticus. In medical literature, reports of Dravet Syndrome include children who have suffered this treatment resistant epilepsy without the availability of current medications. These medications have shown more promise in obtaining seizure control. Although all of the children are born as typically developing, the eventual prognosis for Dravet Syndrome is developmental delay or mental retardation. Fifty percent of the reported children have an IQ of less than 50 by school age. There are many valid explanations for the cognitive deterioration which these children experience over time. These include inflammation along with changes in neurochemicals in the cerebellum, and brain damage from prolonged seizures at the cellular level. Thus, the aggressive intervention at the onset of a convulsive seizure can improve the outcome in children with Dravet Syndrome. Global therapies and enriched environment also improve the outcome. Mortality rates are high due to Sudden Unexpected Death (SUDEP), accidents and injury. SUDEP often occurs in sleep and could be a result of an unrecognized prolonged seizure. Fatal accidents and injury occur often due to the lack of the child’s ability to develop appropriate fear due to their cognitive deficits. Finally, ataxia and atonic seizures increase accident potential.****
JS experiences prolonged periods of rage and anger before many tonic-clonic and absence seizures. He also experiences long periods of obsessive compulsive aggravation that can rarely be satisfied with any action or physical item that he may be asking to hold or obtain. Since October of 2007 he has taken Risperdal, a behavior medication prescribed by a psychiatrist. This summer he started Lexapro for depression. We decided to seek support from a psychiatrist after we were unsuccessful at multiple attempts to implement a behavior plan put together by the Nysonger center the spring of 2007. With all of his energy, JS is a threat to himself when it comes to his own safety. He easily throws himself down on many household items and toys that could hurt him. He frequently kicks and smacks inanimate objects or he will climb to high surfaces, and then jump down. JS also has frequent headaches and mood problems that are associated with his seizure patterns. During these periods of rage, anger, and pain, he frequently tries to hit, kick, jump on, and push his brother RS and other people. Despite efforts to protect RS, both his parents and providers have not been able to keep him safe on many occasions from JS’s aggression. RS is in danger every minute of the day in our home. We do our best to keep JS’s behavior in check. However, he does not respond to most traditional psychologist recommended techniques. When they are affective it is usually for short periods of time until a new technique is quickly needed. Our hope is that with the medication and more assistance in our home that we will finally be able to implement JS’s formal behavior plan. He frequently produces bruises on both of his parents with his aggressive acts of kicking and hitting. He will be completely happy and playing one minute and angry and hurtful the next minute. RS is not an aggressive boy and also has developmental delays. His natural character is rather passive and introverted. He is scared of JS and will frequently yell out to gain attention in advance of his brother approaching nearby. Without JS having one-on-one attention 100% of the time RS is not safe. JS will on occasion enter RS’s bedroom while he is sleeping and jump on him, hit him, poke at his eyes, or bite his face, arm, or leg. We do everything humanly possible to avoid these incidents, but they still happen. As his parents we are only safe because we are adults of average size. JS is extremely strong for his size and we do not believe that he is safe from even himself.
A new phase of acceptance of JS’s chronic and progressive condition has surfaced. We have known for years that he requires twenty four hour intensive supervision and immediate intervention at the onset of a seizure. Our family’s diligence and sense of urgency to his medical needs have saved his life and we are told that we may have spared regression of his skills. Attached you will find a summary of his seizure logs the month prior to starting treatment with Stiripentol (STP) and the month after treatment began. You will notice that his seizures with the medication are drastically improved to date! Without this medication JS would wonder the house in a trance-like state….this is actually a prolonged absence seizure. When a prolonged absence seizure occurs at night we never know where we will find him in our home. He could be in his bed, in the hall way, bathroom, kitchen, family room or on the floor in our bedroom. We have woken up many times from hearing him breath heavy. He will stand there staring blankly at us sleeping. At these times he is unable to talk or even tap us to try and wake us up to help him. Of course it is dark and we are trying to wake up ourselves to turn on the light and check his state of alertness. On another occasion he has tried to let himself out of the house in his altered state of awareness in the early morning hours. We fortunately were alerted by our household alarm system. Nighttime wandering is extremely unsafe… he could fall down the flight of 13 stairs or start to convulse without our knowledge (being out of range of his baby monitor) someplace in our home. Factor this problem in with having another child with nocturnal seizures and the nighttime safety and stress factors are immense.
Currently we are paying out of pocket over $990.00 a month for JS’s anticonvulsant medication, Stiripentol (STP). Because medicine is still awaiting FDA approval we will soon be forced to choose between paying our house payment or purchasing medication for our son. As every parent understands we have no real choice we must sacrifice our home and help our children get better or at least continue to improve their quality of life. Stiripentol is the only medication in the world specifically designed for Dravet Syndrome. Stiripentol is approved in Europe and is a medical necessity for JS. It is part of the “magic” cocktail that seems to help the Dravet Syndrome child gain seizure control. It is only a matter of time before we are forced to lower our level of care for JS. His father is working one full- time job with excellent state benefits and two part-time jobs to help make ends meet. This takes him away from the home for most evenings and weekends. I work freelance when I can currently but need to go back to work full- time especially with the recent increase in energy costs. This choice is not in the best interest or advocacy of JS’s needs. When this happens we cannot be available at a moment’s notice for emergency calls from school multiple times per week or for his after school care ….after 2:30pm. Without the ability to provide skilled nursing care in our absence… our stomachs turn ….just thinking about one parent not being able to leave work while our son is seizing at school or childcare. When we are required to leave a job site multiple times per week we are not likely to maintain the same job for very long. JS’s elementary school only has nursing staff one day per week for a building of nearly 400 students.
Most reports of Dravet Syndrome in the medical literature are of children who are institutionalized because of the burden of care necessary to keep them alive. JS’s diagnosis in December of 2007 of Dravet Syndrome has brought to light the fact that his needs go beyond normal custodial care of a six year old. As parents we have gone beyond the imaginable (for most) to ensure the best outcome for him. We have sacrificed our time, careers, energy, and resources and have maintained advocacy for JS despite the inflicted lifestyle we are enduring. He is able to articulate many words and phrases and at the surface appears to be a somewhat “typical” six year old in his development. However, he scores severe in speech delay due to inconsistency in being able to express his ideas in detail and his difficulty staying on topic to complete his thought. He needs close physical monitoring by OT & PT services as his body changes in strength and coordination from the constant seizure activity. We realize the level of commitment necessary for his survival and best outcome is lifelong and beyond what is humanly possible to do without continued skilled nursing assistance and behavioral supports – first and foremost for JS’s health and safety.
We are exhausted and desire to have more normalcies in our family life. For five years, we have taken turns sleeping in the same room with JS to monitor for a seizure that could take his life in his sleep. Many of his seizures happen while he is sleeping. Many also happen at active play time or during periods of increased physical activity. i.e. the gym at school or on the playground. All of these seizures have required immediate intervention. For at least five years we have not been able to go to church together as a family because no one in the church is capable or willing to care for JS. He cannot sit thru a service because of his behavioral outbursts, fixations/OCD, and perseveration. For five years we have not had time together as a couple to grow our marriage. Our “home” is failing socially, emotionally and financially. Please strongly consider granting JS an Emergency Status IO Waiver with a substantial allocation so that he can have the proper medical and behavior support throughout the day and night. Let us know if we can provide any additional information or materials to assist in the review process.
****Please read more about Dravet Syndrome in the pamphlets submitted with this letter or by visiting the web site www.ICE-epilepsy.org