Fresh into 2018, we are really pleased to bring you our second Warrior of 2018 – Faith,as told by Jenny, who we know has been a massive source of support and knowledge to so many.
It’s funny how the little things can have a massive impact at a stressful time in your life. I had to visit the hospital recently for a meeting with the UKIST medical advisor and the smell of disinfectant in the toilets hit me right in the solar plexus and took me straight back 3 years to the worst day of my life.
My seven month old daughter Faith had started to do a jerking movement – the first few times it happened as an isolated head drop movement in her highchair and we laughed as she got a faceful of porridge and figured she was not quite stable sitting up just yet. At Christmas I had looked forward to handing her over to her doting aunts and getting a bit of a breather, but she was clingy and withdrawn – we thought she had a virus. Looking back at those photos breaks my heart – the light had gone out of her eyes and we had no idea. Just after Christmas she had her first cluster of spasms, jerking her head into my shoulder while I was carrying her. Three days later in the bath she did it again – crunching her belly and flinging her arms forward, resting for a few seconds, then doing it again. This time we caught it on video on a smartphone and sent it to a couple of doctors in our family. Within minutes they were on the phone telling us to take her to A&E, they suspected she was having infantile spasms.
We went in first thing the next morning, and were so lucky that our local children’s A&E is Bristol Royal Hospital for Children, who I now know are one of the big regional neurology centres and were involved in a lot of the IS research studies that have happened in the UK. The junior A&E doctor didn’t know what our video showed, but was concerned enough to call the consultant, who admitted us and ordered an EEG. The technician did not have sufficient poker-face to disguise the fact that the EEG trace was far from normal. We were then visited by a whole team of neurologists who broke the news that the EEG showed hypsarrhythmia – the constant chaotic pattern that is diagnostic for infantile spasms. It was in hindsight a blessing that creaking hospital admin meant I had no access to the internet those first few days and nights, syringing huge doses of steroids into my baby and watching helplessly as she racked up spasms into the hundreds. What information my husband Tim brought in was bad enough, there was very little good news about IS on the internet. There was no UK support group at that time, I was so grateful when I was admitted a few days later to a US-based international support forum.
It’s hard to describe to anyone who has not experienced it how miserable it is to care for a child on high doses of steroids. They are ‘hangry’ – permanently starving, refluxey, wired, exhausted but they can’t sleep. The baby you love disappears behind rolls of flab, they sprout hair in unusual places and they rarely smile. They are immunosuppressed so you can’t even go out for a change of scenery. It is so hard to untangle what is side effects of the medication and what is the disease itself, you are left wondering if this is what the rest of your life will be like. Couple that with the worries about what this meant for our future, and it is no wonder that we all spent many days in tears.
We were extremely lucky that Faith responded after 4 days to the first medication prescribed, and that after a wobble when we first tried to wean and she relapsed, we were able to successfully wean off steroids. She was left with adrenal insufficiency (her adrenal glands shut down during treatment and were not able to recover to the point where they could produce cortisol, which meant more medication to counter that) The battery of tests she underwent did not reveal a cause for her developing IS, meaning she was classed as ‘cryptogenic’ (hidden cause) which bizarrely enough was cause for celebration since outcomes are often better in children with no known cause.
At 12 months old she had been off prednisolone for 2 months and was returned to more normal proportions. Her EEG was much improved though there was some ‘slowing’ which often relates to areas of the brain not functioning normally, but she had regressed during IS and treatment and was assessed as delayed to the level of a 6 month old in all areas of development. We naively assumed from reading the experiences of our American friends that all sorts of early intervention specialists would be lining up to help us, but found that the NHS provision is rather different, months and months go by with nothing being done and thresholds to qualify for therapy are high. At that point we enrolled in a program with Snowdrop for Brain Injured Children, a charity based in Devon which aims to harness neuroplasticity (the power of brain cells to rewire themselves ) to help children with developmental issues due to brain injury. We also cobbled together a schedule of various local groups to try and boost development as much as we could – a monthly Portage playgroup, a Rainbow group at the children’s centre, a local parent-carer playgroup, Sing and Sign, gym, swimming. Every parent who goes through something like this finds their own way to cope, mine was to read absolutely everything I could find about infantile spasms and development delay – the internet forums, scientific papers, and books.
In time, I was no longer the newbie asking questions, but one of the people answering them. I came across other British parents on the international support group, and this led to us setting up a UK based Facebook group and then registering UKIST as a charity. The day the trustees met for the first time at our first AGM was an emotional one. It is one of our key aims to get IS parents together – both online and in person – because support from other families is so crucial. I’m enormously proud of what we’ve achieved in our first year (and it’s not over yet).
At 42 months, Faith is a pretty normal 3 year old apart from being a bit dinky (the steroids stopped her growing). She talks non-stop, can ride a balance bike at top speed, and bosses her big brothers around. She thrives at nursery and is heading for mainstream school in September. If she has any lasting damage from the spasms it looks likely it will be mild. We are under no illusions how lucky we were to have a quick diagnosis and successful frontline treatment giving her the best chance. It is a tragedy that many children continue to be diagnosed late and treated inappropriately, reducing their chances. We’re committed to campaigning for change, for all our little warriors.