HOUSTON – The appropriate evaluation and treatment of infantile spasms often comes after significant delays that are largely attributable to lack of recognition of the condition, according to recent survey results.
“Parental suspicions that ‘something was wrong’ were often discounted by health care providers, and survey respondents frequently reported that health care providers (including neurologists) were unfamiliar with infantile spasms [IS],” wrote Johnson Lay and his collaborators, in presenting results of the recently-completed Assessment of Symptoms and Specialists in Infantile Spasms (ASSIST) study at the annual meeting of the American Epilepsy Society. It’s critically important not to discount parental concerns, senior author Shaun Hussain, MD, said in an interview about the study, which surveyed parents of children with IS: “Sometimes we get in the habit of reassuring patients too much.”
Johnson Lay and Emily Cheng (coauthor)
On average, a pediatrician will see two cases of infantile spasms in a career of caring for children, Dr. Hussain said. These characteristically brief episodes last just a second or so, and frequently come in clusters, during or after which crying is common. Though the classic presentation of IS is a brief drop of the head with accompanying elevation of the shoulders – and perhaps a lifting of the arms – there’s wide variation in presentation. “Infantile spasms can present in a lot of different ways; babies haven’t read the neurology textbooks,” Dr. Hussain said. A curated collection of videos showing the variety of infantile spasms is available on the Infantile Spasms Project website.
Mr. Lay and his collaborators surveyed the parents of 100 children with IS in order to understand and describe their experiences in having their child receive the correct diagnosis and treatment. Also, they sought to determine whether socioeconomic factors affected prompt access to care. Dr. Lay completed the study with Dr. Hussain and other coauthors after graduating from UCLA in 2015.
The study, presented during a poster session at the meeting, measured the time between the parents’ first identification of IS and their contact with an “effective provider.” A provider was deemed effective if he or she correctly diagnosed IS and prescribed a first-line therapy: adrenocorticotropin-releasing hormone (ACTH), corticosteroids, or vigabatrin (Sabril).
Of the 100 children whose families were surveyed, 29% were seen by an effective provider within a week of the onset of IS. The median time from the onset of IS to the patient’s being seen by an effective provider was 24.5 days, with a wide range seen in the population surveyed (interquartile range, 0-110.5 days). This exceeds the 7 days that has been identified as the goal cumulative latency to treatment, Mr. Lay and his coauthors said.
The median age of onset of IS was 6.6 months in the study population, 45% of whom were female.
A total of 64 patients were first seen for IS concerns by a provider who was not a neurologist. Of those, the cumulative latency to seeing the first effective provider was over 100 days, with some patients’ wait time stretching to over 200 days.
The survey asked about socioeconomic factors, including income, health insurance status, proximity to specialty care, ethnicity, and parental language preference. Of these, only a non-English parental language preference was associated with increased time to seeing an effective provider (hazard ratio, 0.37; 95% confidence interval, 0.20-0.68; P = .002).
“These results suggest that a simple lack of awareness of infantile spasms among health care providers may be responsible for potentially catastrophic delays in IS care,” wrote Mr. Lay and his coauthors. Dr. Hussain concurred: “The clear driver of treatment delay is the failure of health care providers to recognize the appearance, importance, and urgency of infantile spasms. Delay in diagnosis and treatment of infantile spasms is common, costly – to both patients and the health care system – and almost entirely preventable.”
The Elsie and Isaac Fogelman Endowment, the Hughes Family Foundation, and the UCLA Children’s Discovery and Innovation Institute funded the study. Dr. Hussain has received research support from and been on the advisory boards of several pharmaceutical companies. Mr. Lay reported no disclosures.
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