Quality of Life and Medication

Enjoying family time in our back garden

Over the past few years medications that haven't suited Chrissy have caused her untold suffering. From general anaesthetics and pre-meds for ops in early childhood to antipsychotics, reactions have ranged from screaming & thrashing about as if acid is burning her insides to status epilepticus and a life-threatening blood disorder. I've no idea why Chrissy is so sensitive & has such strange reactions but trying her on new medications scares me witless. It's unbearable to see the torment she goes through but a relief that while she's in hospital they can respond quickly to remove medications that have caused bad reactions. Out in community-based settings, this did not happen.

When a supplementary antiepileptic caused mayhem in Chrissy's brain earlier this year, I wondered if she would ever be stable again. It was supposed to do the opposite - it's prescribed for anxiety as well as seizure control. So when her doctors started suggesting other adjunctive antiepileptics my hackles went up. Chrissy has been on Epilim alone since the end of April, and has got her sparkle back. She has been interested in her surroundings, engaging in meaningful conversations, able to focus on activities, and a delight to be around. Introducing a new drug would jeopardise her newfound eqilibrium. Was it absolutely necessary?

Chrissy's seizure freqency has increased slightly, and they're mainly complex partials that become generalised while she's in bed at night. They're all self-limiting and last for only a minute or two.

My questions were: Are they likely to damage her brain? What's their impact on her overall quality of life?

We saw the neurologist on Thursday, No, the type of seizures that Chrissy has do not cause brain damage. Hospital staff and I felt that Chrissy's life is briefly interrupted by seizures but not ruled by them. She will sleep for a couple of hours after more major seizures, then will wake up as if nothing has happened. One of Chrissy's nurses asked about the risk of SUDEP (sudden unexplained death in epilepsy) ttp://www.epilepsysociety.org.uk/NewGetInvolved/Awarenessraising/EpilepsyReview/SUDEPresearch. Her neurologist said that no one knows exactly why SUDEP happens but it's more common among people with poorly controlled epilepsy, who have seizures at night during sleep and whose seizures start from a focus then become generalised. So Chrissy does have several risk factors but close monitoring is key. She is in the next bedroom to ours at home and we're attuned to the slightest change in the sound of her breathing, and hospital staff check her every 15 minutes. She also has a baby monitor. I try not to think about SUDEP - everything that can be done is being done and what's more important for Chrissy - optimising seizure control with all it entails or quality of life?

Chrissy came along to the appointment and it was clear to us all that she was on top form. The highlight of the day came as we went to leave the hospital. She did a body-swerve towards WH Smiths, made a bee-line for the sweets then homed in on the Maltesers. There she spent ages bent double over the shelves with her bum in the air trying to work out the difference between two packs - one was slightly bigger than the other & she wasn't going anywhere until she'd made the right choice! The rapture on her face as she tucked the larger of the two packs inside her lunchbox was a joy to see. I felt elated all day as I reflected on how far she'd come.

'Don't count your chickens' Ian warned me later....

I know there are likely to be more blips but for now I'm enjoying the feeling that we've got Chrissy back. If the risks posed by her seizures increase I will, of course, be open-minded about the need for adjunctive treatment but we've all agreed for now that watchful waiting is the best approach.



I am a SWAN UK (Syndromes without a Name) blogger

Epilepsy & Behaviour

Chrissy aged 10, with her chocolatey sister, Alex, 6

Since Chrissy's Topiramate has been swapped with Pregabalin, she's had two clusters of night-time seizures, one requiring rectal Diazepam. This is Chrissy's usual pattern but seizures were infrequent on Topiramate - the price she paid was being over-sedated, though not enough to quell her self-injurious outbursts. When her seizures started up again I hoped she'd get a break from her outbursts. They tend to be less severe during seizure cycles but this pattern has got less apparent as she's got older.

The reciprocal relationship between epilepsy and behaviour is recognised among medical professionals. It's like a pressure cooker releasing steam. Chrissy would have a build-up of challenging behaviours then seizures followed by a brief period of calm. When she was little I thought there was even more to it than that, and became convinced there was an epileptic component in the severe self-injurious outbursts. Chrissy's school teachers felt the same, and described the way 'something just came over her' as she sat doing something that she enjoyed. I became increasingly frustrated that health professionals were dismissing these outbursts as purely behavioural - as if they'd cease in a different environment.

When Chrissy was 10 my frustrations boiled over...

She was in Great Ormond Street Hospital for a routine MRI scan. A general anaesthetic was arranged because she wouldn't have co-operated, & she was given a pre-med. It sent her to sleep but she woke up when she was moved from her ward to the scanning room, and exploded into an outburst. She bashed her head repeatedly against the metal bars of her bed and tore at her skin. 'Now at last her neurologist will see one of these & organise investigations to find out what's going on,' I thought. How naive I was!

The hospital nurses were shocked at Chrissy's distress. One nurse, who'd worked in child psychiatry for years, said that she'd seen children with mental health problems in meltdowns before but this went way beyond that. Normally they gave up when they were exhausted but Chrissy appeared to be in the grip of something beyond her control.

The neurologist was called to the ward. He appeared, flanked by junior doctors, took one look at Chrissy, who was thrashing around on the floor by that point, and said: 'That's behaviour.'

As he turned on his heel to walk away, red mist descended & I threw questions at him like darts: 'If it's behavioural then how come she's had these from infancy? Why do they wake her at night? Why are they triggered by general anaesthetics & some medications? Why doesn't any intervention stop them? Why are they self-limiting? Why are they cyclic and worse in late afternoon? (as they were then) What about the strange physical symptoms - complaining about sore eyes, clawing at her face and extremities as if they hurt? What about the reciprocal relationship with the seizures?'

The neurologist insisted: 'What we're seeing now probably isn't the same as those you saw when she was a baby. This doesn't look like any seizure that I've seen. Epileptic episodes don't last as long as this. They're behavioural.'

I stormed off and had a good cry.

When I calmed down and returned to the ward, the neurologist took me into a side room and explained: 'I doubt that what I observed is caused by epileptic activity but I agree that there appears to be a reciprocal relationship between the seizures and behavioural outbursts. We really don't know what they are but behaviour sometimes worsens in children with epilepsy when seizures were well controlled.'

He decided to try a new anticonvulsant - Gabapentin.

Almost immediately Chrissy slept through the night for the first time in weeks. Two days later, the first thing I noticed when she came home from school was her smiley face and pink cheeks. (She looks pale & wretched when she's going through an outburst cycle). I asked her for a kiss. 'Screaming's gone.' she said.

Her teachers reported that she'd been 'brilliant,' amenable and well-behaved at school. I felt ridiculously emotional. Surely it was too early to be Gabapentin? Maybe it was a delayed reaction to all the pre-op sedation & general anaesthetic?

In fact, it was probably Chrissy's emergence from another bad cycle of outbursts but, when she's well, I always feel giddily optimistic that it will last. I rang friends and family to tell them about the change in her. 'Don't count your chickens....' they gently warned me.

Taken from my book: 'Bringing Up a Challenging Child at Home.'



I am a SWAN UK (Syndromes without a Name) blogger

Small Steps - A #Special Saturday Post

Over the past few weeks Chrissy's affairs have taken precedence over everything else - but it's been worthwhile.

I had a positive meeting with her new psychiatrist & was very impressed that he'd taken the time to read this blog! He referred to incidents I'd written about & it was clear he'd researched her chromosome disorder. He was open-minded and interested in exploring causes of the more intense clusters of self-injurious behaviours for which no environmental trigger has been identified. As Chrissy had settled down after a difficult month, he decided to withdraw the last 15mg of her Topiramate while introducing Pregabalin. I don't know if that's why we had such a challenging time with her. Her last spell of horrendous behaviour coincided with Topiramate withdrawal & it was similarly intense here yesterday. Her diva-ish behaviour has been off the scale - she's been moaning, obsessive, demanding & irritable. We did have a lovely cuddle with her on the settee while we watched Come Dine With Me but had to hold both her hands the whole time, effectively handcuffing us to her. A great way of getting our individed attention! She then kicked off again later & wouldn't go to bed until after midnight.

I'm worried about how Chrissy will cope at Xmas, particularly as last year was so awful. She will obviously be here with us on Xmas Day, as she's always been, but when more of the family visit on the 28th it could be apocalyptic. Her stripping is particularly difficult to manage when there are young men & children around, as you can imagine!

Anyway, on a different note, another positive outcome came from the 1st stage of our appeal process re the long-standing funding dispute between the local authority & NHS trust. The dispute has caused a number of problems that I can't elaborate on publicly at the moment but our biggest recent concern was that no one was taking care management responsibility. We need to plan for Chrissy's discharge from hospital & the type of 'bespoke service' she needs may, according to estimates from the Challenging Behaviour Foundation, take about 12-18 months to organise. So, although she needs to stay in hospital until meds changes are completed, we need to start planning now.

At our appeal meeting the NHS trust admitted to 'horrendous' errors in the healthcare assessment process, & offered to start from scratch with a retrospective assessment. This means combing through Chrissy's records from the past three years. I was advised that to see those records, I'd have to apply to the Court of Protection for Deputyship, which puts my responsibilities for Chrissy on a more legal footing. I filled in all the forms, so I could make decisions re property as well as health & welfare, as she may end up with a home of her own. The forms are tedious & repetitive, & took me nearly a day to complete but they're done now. I'm sure it will be worth it in the long run!



Please join the cause by joining the facebook page - http://www.facebook.com/SpecialSaturday
Following on twitter - @Specialsat and retweeting hashtag - #specialsaturday
Reading and following the Special Saturday Blog - http://specialsaturdayhttp://www.blogger.com/img/blank.gif.org/home/