March is National Save your Vision Month – and vision is a hot topic for families of children with infantile spasms for two reasons;
Firstly, Vigabatrin, one of the first line and most effective treatments for infantile spasms, has the potential to cause toxicity to the retina and permanent loss of peripheral vision. A diagnosis of infantile spasms often comes as a huge shock to the family and given the ongoing damage to the child’s brain, decisions about treatment need to be made very quickly. We regularly hear from families who have refused vigabatrin based on a misunderstanding that it ‘may cause blindness’. In fact, the medicine has a cumulative toxicity and the risks increase with longer courses of treatment. Most children with infantile spasms will be prescribed vigabatrin for around six months, and the risk of damage during that period is around 5%. This image has been helpful in allowing families to understand that the damage is limited. Vigabatrin combined with steroids has a 75% initial success rate in resolving infantile spasms.
The second reason is that many children with infantile spasms will develop a degree of cortical visual impairment (CVI) as a result. This happens when the eyes work normally, but the visual pathways in the brain are damaged, meaning the brain can’t process input from the eyes. It’s very common for parents to notice pre-diagnosis that the child seems to not be able to see and for some children that difficulty persists despite treatment. 16% of children with a diagnosis of CVI previously had infantile spasms – but there doesn’t seem to be any research about how many children with IS develop CVI as a result, which is part of the lack of data about longer term outcomes that UKIST seek to address. Cortical visual impairment is a condition that can improve with therapy as the neuroplasticity (ability to make new pathways) of the brain can help children develop alternative visual pathways.
UKIST has a video about CVI at https://www.youtube.com/watch?v=ZUo4yfpOfSo and we’d also recommend the site http://littlebearsees.org
