Ella’s Story Ella was a healthy baby until the week before her first birthday in January 2003 when after several days of illness and misdiagnosis by doctors she was admitted to St George’s Hospital with pneumococcal meningitis. She was sedated, ventilated and spent a week in Paediatric Intensive Care. We spent a very anxious week (and Ella’s birthday ) sitting by her bed. The nurses were very positive, and told us that once the infection passed and the sedation was decreased children often sat up as right as rain. Not the case with Ella though, after a week she came around looking groggy and totally floppy. She did not respond to our voices and we instantly suspected she had lost her hearing. Whilst still in hospital Ella had her hearing tested and our suspicions were confirmed, Ella was profoundly deaf.
We were devastated but were told that sometimes hearing was regained after meningitis. We spent a further 3 weeks on the Infectious diseases ward with Ella like a rag doll barely able to even feed from a bottle. Brain scans showed a tiny patch of damage in an area which related to the control of her left side. Ella was eventually sent home having been told “She’ll probably get better but we have no idea how much”. Over the next few months Ella had to relearn everything – lifting her head, and then rolling, controlling her limbs, then sitting up and beginning to crawl again. But her hearing didn’t return. Ella had her first hearing aids in April. However, it soon became obvious that she was getting nothing from her aids, and she was assessed for a cochlear implant at GOSH. Ella had her implant on July 12th, just 6 months after her illness. This was also the day before our next baby (Alfie) was due – fortunately he held on for another week!
After 3 months Ella was responding to sound, turning to her name and she had also begun to walk although she had balance problems. We felt quite positive about her recovery. However, over the next few months, comparing Ella to other children her age, it became obvious that she had been left with learning difficulties in addition to her hearing loss. It was difficult to develop Ella’s hearing through games and activities as she had poor concentration and was struggling to relearn basic play skills. She also began having strange startle episodes, where her arms would extend and stiffen, like a baby’s startle reflex. One day her portage worker saw one of these and said it looked like a seizure.
Ella the day of her implant’s “switch on”
It was confirmed that Ella had epilepsy just over a year after her meningitis. By now the seizures had developed and seemed to be in response to sudden appearance of people and objects – she was quite literally ‘startled’ by them. She would have a tonic seizure, her limbs stiffening sharply, and quite often fall to the ground – fortunately she tended to fall backwards onto her bottom, although she did manage to bang her head frequently, a danger to her implant. She would seem dazed for a little while and then pick herself up and carry on. After a few fits close together Ella would have to go to sleep.
An EEG showed that in addition to these startle seizures Ella’s brain waves were very abnormal all of the time. This would undoubtedly be interfering with her development and could account for her difficulty in concentration. So we began with the first of a long list of Anti Epileptic Drugs which over the next couple of years would not The all too familiar effects of steroids control her seizures but give her a range of side effects. We tried epilim, clobazam, topiramate, ethosuximide and a course of steroids.
The all too familiar effects of steroids
At her worst she became sleepy, unresponsive and like a zombie. We only realised looking back at photos and videos that she had stopped smiling and laughing. Ella’s fits were still no better. It was very stressful, trying not to startle her coming into a room. On many occasions I would peek in and see her in some precarious position, like balancing on the coffee table, and be too scared to come in, in case I triggered a fall. I tended to take her with me wherever I went around the house so as to minimize the risks – even to the toilet on many occasions! Going out was tricky as a car suddenly appearing round the corner would trigger a fit, or someone appearing from behind playground equipment at the park. Ella was at constant risk and needed someone with her all waking hours!
At 3 Ella started school in the Early Years class at Paddock School in Putney – a school for children with Severe Learning Difficulties. Of all the schools I had looked at it was the only place I felt I would be confident leaving Ella. There was a class of five children with a range of conditions, and 4 had epilepsy. The staff were so un-phased by Ella’s complex needs and couldn’t have been more caring and supportive. They developed a warning system for Ella whenever someone was coming into the room, found out all they could about implants (including how to put it back together when Ella had dismantled it!) and took on all advice.
Ella wearing her helmet –
modified to allow for her implant
Ella’s audiology appointments confirmed she was hearing across the range of speech sounds, but her progress was slow. By now it was evident that her learning needs were surpassing those caused by her lack of hearing. The combination of being unable to sit still, to hear in a natural way and the interruption of regular fits, and the sleeps after these, meant progress was very limited. Then shortly after her 4th birthday her seizures stopped spontaneously. Ella’s development picked up, she began to babble for the first time, her eye contact and response to sound improved greatly. She was much more interested in what was going on around her. She learned how to use simple toys, so for the first time she could actually ‘play’ independently – even if only for short periods of time. She was steadier on her feet and her coordination improved. It also meant less stress, without the fear of triggering a fit we could relax a little and allow Ella a little more independence. Around this time we had an appointment at GOSH for the Ketogenic diet trial. As Ella was doing so well at this point, we did not pursue this.
Ella aged 4, seizure free (briefly)
and enjoying Godstone Farm.
We moved house a couple of months later, and found Ella another great school, Brooklands in Reigate. Still on good form and seizure free, the staff couldn’t believe the little girl who arrived was the one they had been expecting. Ella could sit briefly and play with simple toys, and on the first day spoon-fed her own yoghurt!! However, over the next few months the seizures crept back, with the same deterioration in concentration and behaviour. Having seen the difference in Ella when she was seizure free, we were keen to try anything to help her achieve this again. We approached GOSH about the ketogenic diet again. We were lucky to be able to be considered as Ella was still on the original list for the trial , and shortly after Christmas 2006, Ella began the Classical diet at a ratio of 3.5 :1
We had the usual traumas of meals not turning out as expected, forgetting to add vital ingredients, and Ella’s refusal to eat them after our many hours of preparation, but signs were positive from the outset. Within days she seemed calmer and began sleeping better. In January she moved into the reception class at school and went full time. Her concentration and focus improved, once again she was like a different child (still a whirlwind, but able to learn). People at her 5th birthday party watching her running around laughing couldn’t believe she was the same girl who spent most of her 4th birthday party zonked out in her pushchair after several fits. After 6 weeks on the diet her seizures stopped! An EEG after 3 months showed that, although by no means normal, Ella’s brain activity was much calmer. What great timing too – her new brother Harry had arrived on the 19th February- another feisty redhead – just what we needed!
When Harry met Ella
At this stage, we began to wean her off her remaining anti epileptic drugs and began to see greater and greater improvements. As the medication lessened, Ella seemed to come out of a fog and begin interact more with what was going on around her. She was more alert and responsive, and began to play with toys which she had previously shown no interest in. It was as if she suddenly understood what things were for. She would pick up a key and put it in lock, try to put her own shoes on, or to struggle into any item of clothes she could find. She remained drug and seizure free for about six months when we noticed tiny startles creeping back. At this time she was diagnosed with premature puberty and had to begin monthly hormone injections to halt this. The hormones seemed to be interfering with the diet although her ketone levels remained stable throughout and we continued to see the benefits of being drug free. We didn’t change anything, just hoped that when her hormones were regulated the seizure control would improve. Over time things calmed, to just the occasional blinky episode, or flush, and Ella spent the last summer holiday without even a twitch.
When at her best like this she is calmer, more responsive and affectionate. The difference in her from being seizure and drug free is incredible. Whilst her seizures were nowhere near as severe as many other children, the effect of regular seizures and the constant subclinical activity was debilitating. The drugs simply dulled down her ability to respond and interact with the world around her. She continues to be hard work, and her progress will always be slow and erratic, but she is so much more aware of what is going on around her and understanding what is expected of her. She is understanding so much more in terms of speech and can respond to simple instructions and requests and identify people , animals and objects in pictures when asked to. She still has no spoken communication but is much more able to make herself understood – leading us to things she wants, taking our hands to point for things and using PECs symbols to make choices and to ask for things.
She is so much more engaged with us, affectionate and interactive, rather than being engrossed in her own little world for most of the time. Ella is operating at round the 18 month level but she has learned so many simple things since beginning the diet, and like many Matthew’s friends families, the smallest thing is cause for celebration – learning to turn a rain stick, to bounce a ball, to swing herself on a swing, to jump into the swimming pool.
The diet has given her back the potential to learn simple things like this, which other parents would take for granted and we can’t thank Emma and Matthew’s Friends enough for all their support. There is absolutely no way we could have done this without you!
Ella raised over £1000 for Matthew’s Friends
on her Step up Challenge in September 2007.
Update : In Sept ‘08 Ella’s last EEG was normal with no signs whatsoever of epileptic activity !