Appeal type: Medical necessity
Decision: Overturned

Diagnosis: Autism Spectrum Disorder, Medically Refractory Epilepsy; Dravet's Syndrome
Treatment: Additional 14 Hours per Week of Personal Care Services
The insurer denied the additional 14 Hours per Week of Personal Care Services.
The health plan's determination is overturned.

The patient is a who has a sodium channel alpha1 subunit gene (SCN1A) mutation resulting in Dravet syndrome. She has medically refractory epilepsy with predominantly myoclonic and absence seizures. The patient has cognitive impairment, autism spectrum disorder, hyperactivity and gait ataxia. A letter from her pediatric epileptologist, describes that the patient is able to walk, but she has poor balance and impaired judgment. As a result, she tends to place herself in unsafe situations. She grabs items that could injure her, climbs on objects, tries to elope, and puts things in her mouth that could cause her to choke. Her seizures can happen at any time and place her at a risk for falls and injury. Her guardian is her uncle and his spouse.

The patient's seizures began at the age of 9 months with an episode of status epilepticus requiring intubation. She has a history of multiple seizure types, including generalized tonic clonic, focal, absence and myoclonic. Her major seizures stopped after Fenfluramine was started, but she continues to have absence and myoclonic seizures. The patient has behavioral challenges, including significant tantrums with self-injurious behavior. She has apparent relative insensitivity to pain. She attends school with a one to one (1:1) aide and a nurse on the school bus.

Her medications include Fenfluramine, Divalproex, Clobazam, Zonisamide, Clonidine, and rectal Diazepam for prolonged seizures. She also uses MiraLax for constipation, and Ketotifen eye drops, and receives Benecalorie and Kate Farms Standard liquid as nutritional supplements. She has been prescribed oxygen via nasal cannula for use during seizures. The patient's examination is significant for constant movement, and a slightly wide-based gait.

The health plan, in its determination of medical necessity, did not act reasonably, with sound medical judgment and in the best interest of the patient.

The additional requested 14 hours per week of personal care services are medically necessary for the patient. The patient has Dravet syndrome. She has medically refractory epilepsy with predominantly myoclonic and absence seizures. These seizures place her at risk of falls. The reviewer has cared for patients with myoclonic seizures who have sustained facial lacerations, broken teeth and fractured limbs due to falls as a result of myoclonic seizures. She needs a supervising adult within arm's length during waking hours to decrease the risk of injury. The patient has cognitive impairment, autism spectrum disorder, hyperactivity and gait ataxia. She is able to walk, but she has poor balance and impaired judgment. As a result, she tends to place herself in unsafe situations. She grabs at items that could injure her, climbs on objects, tries to elope, and puts things in her mouth that could cause her to choke. Constant 1:1 supervision is medically necessary.

Dravet syndrome is one of the catastrophic epilepsies of childhood. Connolly [1] notes that development is usually normal in the first year of life, but most affected individuals will eventually develop intellectual disability. The typical presentation is characterized by hemiclonic or generalized clonic seizures triggered by fever during the first year of life, followed by myoclonic, absence, focal and generalized tonic-clonic seizures. Non-convulsive status epilepticus and epileptic encephalopathy are common. Dravet syndrome is associated with mutations in the SCN1A gene in 70-80 percent (%) of individuals. The SCN1A mutation results in inhibition of the Gamma-aminobutyric acid (GABAergic) inhibitory interneurons, leading to excessive neuronal excitation [1].