Ivy-Rose’s story is written by her mum Kylie
My beautiful Ivy-Rose was born November 2021. Pregnancy was fine until told I had polyhydramnios and couldn’t go into labour naturally so had to be induced at 38 weeks. Having two previous pregnancies with natural births I was a little nervous. I ended up with covid when going in to have her and had some complications which led to an emergency section where I lost 4 pints of blood and Ivy had to be given oxygen, have fluid drained off her lungs and antibiotics through IV to prevent infection. It was a tough start and we were alone but from then she was absolutely fine and probably my most chilled baby. She was happy and developing as expected.
Ivy was 10, nearly 11 months old when I noticed her first cluster of head drops. I thought nothing of it as babies do quirky things but when she did it again a couple of days later I decided to use google (I know that’s never the best idea) but it did tell me it can be epilepsy related. I had no idea IS existed until then and with me being on anti-epileptic medication, I recorded the episodes and took Ivy-Rose to the GP, October 2022. She looked at the videos and said it looks nothing like epilepsy but with my history and my level of concern she said she would refer to paediatrics. These episodes continued daily and more often so a few days after her 1st Birthday when they were over 5 mins long multiple times a day, I took her A&E. We were in a couple of days and they did an EEG which she was awake for and really fidgety so he said I think they may need to do another while she is sleeping. They discussed this then decided against it and sent us home as the EEG was clear and we were told it wasn’t IS. Two days later the episodes were lasting 20 minutes, also by this time she had regressed in her communication, no gestures and no words like she was doing. I took her back to A+E and was made to feel stupid for taking her and said they are myoclonic seizures that are harmless and happening more due to her having covid and bronchiolitis, but would arrange another EEG. I wasn’t overly happy but thought they know what they’re doing (I didn’t know about UKIST then or the protocols). January was the next EEG which came back clear but I still wasn’t happy with the episodes and the regression and rang the consultant office and epilepsy nurse a few times a week, every week and eventually got another EEG in June 2023, now 10 months since the first episode.
I knew there was something wrong, my happy smiley baby who was progressing had regressed, being constantly tired and cranky with awful sleep but I still thought the EEG would come back clear. I only ever had short EEGs. She went the Friday morning then just before tea time I received a call from the consultant that it was in fact IS and I needed to bring her in ASAP to start treatment. I couldn’t believe what I was hearing after all the months of being told it wasn’t and me having to constantly try to contact them and ruling IS out, it was what I had been dreading. I had just arrived in London to celebrate my daughter’s 16th birthday, I burst out crying on the train, at this point I didn’t know what the treatment plan was or what would happen.
She started prednisolone and vigabatrin , had numerous bloods and urine tests and and MRI scan. Luckily the MRI was clear and we are still waiting for genetic results. My poor baby was like a zombie and then they wanted to increase the dose which I held off because of how lifeless she was. After a few days she came around more but wasn’t herself and her blood pressure was high and heart rate low so she had to be monitored, the bp remained high even after weaning pred. After 8 days we were able to go home, I was so happy to get home and see my other girls but so anxious as I wouldn’t have the doctors and nurses to make sure my girl was ok. I can’t fault the staff on the children’s ward at Royal Stoke UHNM. The pred made her gain weight, to the point she was almost unrecognizable and her sleep was awful. Luckily by this time I had discovered UKIST and was able to share my concerns and realised I wasn’t alone. Many others have been through this and they reassured me the side effects she was experiencing were normal. I finally had people to lean on and they have educated me more on IS than the medical professionals – the support and quick responses from the team is amazing. Honestly don’t know what I would have done without them.
Ivy-Rose will be two on the 2nd November and has come so far already. She weaned pred and we began to see smiles again and she started to notice things where before she seemed oblivious to everything. The team are still amazed as to how she learnt to walk whilst having undiagnosed IS and think she has definitely broken the mould of textbook IS. Her communication still isn’t there yet, she’s started to babble again and clap hands but nothing else as of yet but when these little milestones happened again it felt incredible and I feel so lucky. I know every child has their own difficulties with IS and each one of them are incredible, strong little warriors. I have appointments coming up as I’m concerned about some movements and have been since a couple of weeks after pred (still on vigabatrin) her sleep is awful and her temper has really upped with pinching, hair pulling and biting but that could be due to frustration of not being able to communicate.
I feel more confident getting what is needed for my daughter with the knowledge and guidance from UKIST. I am incredibly proud of Ivy-Rose and will continue to fight her corner. I know it’s still quite early for us to know any outcomes but with this wonderful group, I know I’m not alone. All I advise for other parents is to go with your gut even when professionals tell you different and push until you get answers. I know this charity will do everything they can to make sure babies like mine don’t have to wait 10 months for diagnosis. Thank you again 
UKIST successfully campaigned to have infantile spasms recognised as a special case in the epilepsy diagnosis guidelines. The 2022 revised NICE guidelines say that any infant suspected of having IS should be referred to a specialist within 24 hours and have an expedited EEG. A 30 min recording is standard for a first EEG but this does not always show the characteristic chaotic patterns of IS, so where concern remains a long recording that captures sleep and the concerning episodes should be performed. UKIST continue to campaign for this standard of care to be offered to every child.
