Our Warrior this Week is the beautiful Benji, here Lisa, one of our fabulous UKIST trustee’s tells you all about Benji’s story.
This is our lovely 17 months old Benji. We had a little bit of a complicated pregnancy with Benji (he had too much fluid on his brain at our 20 weeks scan) so when he made a safe arrival 4 days early we were so relieved to see he was a perfectly happy and healthy little boy.
4 months past with no problems or health concerns besides being extremely colicky. On the Friday we took him for his routine jabs ( we don’t believe this is what caused them now knowing about Infantile Spasms) on the Sunday morning we woke up normally and bathed Benji, when I started to get him dressed he started doing these very subtle eye rolling movements so I called in my husband -to be honest we thought maybe he was having a convulsion after his jabs on Friday having never seen a convulsion but having it run in the family. We carried on our day and they continued most people seeing them thinking he was just looking up. I wasn’t convinced as his eyes were also streaming. So the next morning I took him to our GP, as soon as I started to explain what he was doing he very quickly shut me down and said these aren’t seizures he has fatigue.
He had one in the GP’s room and he still said it was fatigue. So I took him to my mums (they’re very close and she’s had 4 children so I thought she would have a better idea, I needed someone to reassure me I wasn’t losing my brain) these continued so I took him to a&e with a video that we’d managed to catch.
The a&e staff were very nice checked him over and said he was fine but when we showed them the video they agreed it didn’t look ‘normal’ so fitted him with a canula and admitted him. That night they progressed becoming more frequent and his left arm started to move up in a short quick motion. We saw 3 Dr’s that night that said some babies do this and it’s completely normal at the same time as the nurses seeing and saying they looked like seizures to them. By chance a neurologist was in that night and had heard of IS before. He came straight in the next day and viewed the video. He said to us he had a feeling of what it could be but tests would need to be done ASAP, (By this point his legs and arms were flying upwards and his back was curling backwards) So the next day we were booked in for an EEG.
The Royal Alexandra children’s hospital in Brighton doesn’t have the facilities for an EEG so we made a short trip to a neurological hospital were he had his first EEG. The lady doing the EEG had a look of panic on her face and quickly went to get another member of staff. We had the short EEG and were told to go straight back to the children’s hospital which we did. We walked into the ward and the neurologist was waiting in our room. He gave us the diagnosis of IS/West syndrome and started him that day on prednisolone. The next day he was nil by mouth and booked in for an MRI and lumbar puncture. The sedation given didn’t work so our poor Benji had the lumbar puncture wide awake at this point myself and husband were asked to leave but my mum stayed with him. We could hear him screaming outside the hospital and this was by all means the worst part for us and we just sobbed.
We had been given a booklet but struggled to find any information on IS and the information we did find had very poor outcomes. We were later discharged and pushed through the drama of the steroid effect. The spasms stopped within a week. A month later his eyes started to roll again, within hours we took him back to hospital where his neurologist already had a plan set up, and another EEG was performed. This time the hyps were not on his EEG so the video was sent to a neurologist that specialises in IS at the Evalina hospital she agreed they looked exactly the same but decided to trial him on pyrodoxine, our neurologist is very good and takes our concerns into account so we made a joint decision that within 2 days we’d go for the prednisolone again which again worked very quickly. By Christmas his eyes were again starting to stream and the decision was made quickly to put him on vigabatrin. This worked immediately and spasms haven’t been seen since.
Benji has some developmental delay and 2 months ago stopped the vigabatrin. We almost immediately saw a difference in him he’s so much more alert he’s quick at learning etc. In the last 3 weeks he’s made tremendous progress he is finally making baby babble noises, sofa surfing etc. He has some autistic tendencies so there is question as to whether he has autism. We don’t think he has we would go more along the lines of him just being delayed but only time will tell. His motor skills are poor and he’s still so behind in speech and language he is just waiting for his appointment at the development centre. We have however paid to see a speech and language therapist who has said although he’s delayed he does have the basic skills to learn and he is a quick learner. He is a completely happy little boy currently with the funniest personality. He’s very loved by all who meet him. Though sleep is still horrendous more like a newborn he’s had to be weaned on to food various times due to him being funny about textures. He’s still on sodium valproate and cloneazapam. But everyday he shines.
We are truly thankful to Benji’s neurologist for listening to us and always giving Benji the quick treatment that he needs to carry on progressing. We feel very lucky to have a neurologist that was is aware (Benji was his first case and he’s seen many others since). If it wasn’t for him we couldn’t even begin to imagine where Benji would be now.