Abstracts

Rationale: Limited data is available regarding the magnitude and effect of treatment lag on ACTH or steroid response in children with West syndrome from the Indian sub-continent. Also,applicability of studies from other regions is questionable considering the varying etiologies, different therapeutic protocols and access to health care services. Methods: We conducted a prospective study in a tertiary care hospital in north India between January ?" December 2014. A total of 82 consecutive children with West syndrome were enrolled. Magnitude and determinants of treatment lag were determined in all the children. The treatment lag was calculated as the time delay between onset of spasms to the initiation of ACTH/ steroid therapy in days. This time period is inclusive of the time taken to make the definitive diagnosis which was considered as the diagnostic lag period in our study. Short term therapeutic response was taken as cessation of spasms within 14 days of therapy and sustained for a period of ?- 28 days from the last witnessed spasm. To ascertain the effect of treatment lag on therapeutic response, we excluded fifteen children who had either received therapy for less than 2 weeks or had received vigabatrin. We analysed following factors: age of onset of spams, etiology, treatment lag and gender for their association with therapeutic response. Results: Of 82 children, 2 had extremely high treatment lag and appeared as outliers. Of 80 children, the median diagnostic lag period (time taken to make the diagnosis from the onset of spasms) was 37 days (95% CI: 56-121 days). Median time duration between diagnosis and initiation of therapy was 30 days (95% CI: 38-97 days). The total lead time to treatment was then calculated that had a median duration of 90 days (95% CI: 110-198 days). The total treatment lag was sub categorized into 5 time duration intervals as follows: 0- 30 days, 31- 60 days, 61- 90 days, 91- 120 days and > 120 days. There were 14 children in 0- 30 days, 15 children in 31- 60 days, 11 children in 61- 90 days, 8 children in 91- 120 days, 34 children in > 120 days interval. Of the two outliers, one child had a diagnostic lag period of 90 days and total lead time to treatment of 1185 days. The other child had a diagnostic lag period of 1060 days and total lead time to treatment of 1116 days. Only 29% (n=24) of parents considered initial spasms as seizure and sought medical attention at once. When one/ both parents were graduates, the median duration of treatment lag was significantly shorter compared to the duration of treatment lag when neither of them were a graduate (87 vs 167 days, p=0.019). In children with normal development prior to spasms there was a significantly shorter lag period to treatment when compared with children who had developmental delay (43 vs.133 days; p value of 0.006). The duration of treatment lag varied considerably when different groups of physicians were consulted and had statistical significance (p=0.001). We analysed four factors: age of onset of spams, etiology, treatment lag and gender for their association with the therapeutic response. Out of these, our study showed only a shorter treatment lag was associated with a better spasm cessation rate (p = 0.011). Age of onset of spasms, gender and etiology did not affect the treatment response as evidenced by the cessation of spasms Conclusions: We observed a long lead time to treatment (median=90 days) compared to the developed countries. The major underlying factors of the time lag were: awareness and educational status of parents, qualification of the initial practitioner visited and pre-existing developmental delay in the study population. Of all the factors studied, only a shorter lead time to treatment had a favorable association with the therapeutic response (p=0.011). Funding: none