Approximately 0.0064% of the United States population has a disorder called Dravet syndrome, a devastating disorder for young children with used to be known as severe myoclonic epilepsy. According to the Dravet Foundation (2018), approximately 80% of infants diagnosed with Dravet syndrome have a present genetic mutation SCN1A (https://www.dravetfoundation.org/what-is-dravet-syndrome/). Unlike other genetic disorders, children do not have to have this mutation in order to be diagnosed with Dravet syndrome. What makes this disorder so damaging to a child's health and general function are the numerous seizures that occur during infancy.
Multiple types of seizures occur before the age of 15 months and can continue throughout a child's life in varying severity and timing. Often-times, a child will seem to develop quite normally before the age of 4; however, due to increasing seizure activity of the brain, the child's development starts to slow down. Depending on the area and extent of brain damage, children with Dravet syndrome may experience difficulty with walking (abnormal gait patterns), intellectual disabilities, behavioral and emotional issues, and speech or language impediments. Children with Dravet syndrome are also more prone to infections, continuing seizures, feeding problems, and sleep difficulties (Dravet Syndrome Foundation, 2018).
Many children with Dravet syndrome survive into adulthood but require lots of assistance from family and caregivers to meet daily needs. Approximately 10-20% of children with Dravet syndrome die due to a higher risk for sudden infant death syndrome (SIDS). Medications and intense clinical treatments are available in order for children and adults to better cope with the symptoms and to increase their quality of life. Pediatric occupational therapy is among the many services available, but what exactly can this profession do for these children and their families?
In 2015, the American Journal of Occupational Therapy recognized a study that captured the story of a family living with Dravet syndrome. Breland et al. (2015) relayed how the family was able to thrive while caring for a family member with Dravet syndrome, focusing on occupation-based activities in the home. In other words, as rare as Dravet syndrome is the occupational therapy world knows about the condition and has intervention ideas for those affected as well as their family members/caregivers.
Here are some example intervention approaches that pediatric OT may use depending on the child's individual needs:
- Cognitive intervention: Seizure activity in Dravet syndrome frequently results in cognitive damage ranging from mild to severe. This can negatively impact a child's behavior, attention reasoning skills, memory, and so on. Such cognitive deficits affect the child's ability to do anything and everything including activities at home, academics, building relationships, etc. The OT would have to conduct an extensive evaluation to target what areas of cognition the child has trouble with, and then introduce interventions to address the problems.
- Sensory integration: A handful of children with Dravet syndrome may exhibit sensory processing disorders, which should be thoroughly assessed by a pediatric OT with sensory integration training.
- ADL (activities of daily living management): A pediatric OT may introduce ways to modify a child's ability to feed, dress, and bath themselves more independently. This could mean introducing specialized therapy equipment or adaptive equipment for the child or parents to use.
- Functional mobility: Gross and fine motor skills can be impacted as well, which means the child may display challenges with picking things up like a fork or walking across the room. OT can implement therapeutic exercise programs, manual therapy, and other muscle-working protocols to strengthen the child.
- Orthopedic interventions: Some children may require bracing or splinting programs to assist with movements. This could be braces that assist with weak muscles or braces that prevent a child's muscles and joints from tightening up, further restricting movements.
- Family/caregiver education: Of course, the child's OT intervention will include family and caregiver education in order for loved ones to carry out therapy interventions at home with their child.
- Breland, H., Hoffart, A., McDonald, A., Owens, L., Zeigler, J (2015). Who do we live with a chronic condition without expectations? A family's story of thriving with Dravet syndrome. American Journal of Occupational Therapy, 69, doi:10.5014/ajot.2015.69S1-PO5103.