Beth started having infantile spasms at around 7 months of age.  Again it is not uncommon in children with lissencephaly for seizures to change type over time.

The spasms were even more concerning to us - her whole body clenches up during them, her arms generally come into the centre, bent and her legs draw up as well. Often there is also a little cry at the beginning as even her stomach tenses and expels air out a little, but the clenching only lasts a second or two, then it unclenches for a few seconds, then it clenches again.  We also noticed a little tear usually appeared from Beth's eye after a number of these spasms, which made us very sad as it showed she was in a little bit of pain from the effort of this clenching and unclenching.  She didn't cry afterwards, but was very tired.

We discussed with our paediatrician and neurologist what our options were, and basically they gave us two - vigabatrin - a preventative drug that is apparently quite effective against the spasms, but it does have some long term side effects including peripheral vision impairment.  The other option was a oral cortico steroid - prednisone, which has a high dosage for a short period, which can stop the spasms entirely, and then you wean off the medication.  It may not be effective at all, or only for a short time, but it can hold them at bay for sometimes up to years.  There is a similar treatment called ACTH but it is a more heavy duty version and is not oral, but given by injection.

The other benefit of the prednisone is that it may also assist/control the absent/staring seizures and we may be able to reduce or remove the other medication as well, which we would like to do.

Based on this we asked for and were given copies of clinical studies of prednisone vs vigabtrin treatment of infantile spasms in the UK.  After reading this we decided to put Bethany on a similar reigeme and see how she responded.  I will include details of this study on the website soon.

We are now back home and weaning Beth off the prednisone, and we have had  positive results.  She is now only having extremely short spasms 1-2 minutes and only one a day or less.  We are hoping this will reduce further.  We have also been able to reduce the phenobarbitol by approx half without any recurrence of the other seizures.    

She also had another EEG while in hospital this time which they compared to the original one.  They didn't really say much about it except that it did confirm the infantile spasms, which was pretty obvious.