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!



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#Special Saturday post - Groundhog Day

My life feels like Groundhog Day – we've been here three times in three years. Chrissy’s escalating behaviours, placement not meeting her needs, with the backdrop of the funding dispute. Apart from last Sunday's completely nuts bender, which I think has kept me going through this week, I can still focus on little else. Chrissy's spate of awful impulsive/compulsive self-injurious behaviours (SIB) is continuing. (I'm not going to call them outbursts or meltdowns anymore these have a different, far more intense quality). When I look back at the short phone videos I've been taking to show her doctors, I can't bear to watch or listen for long. She is wretched in them, as if she is being tortured by something outside her control.

These behaviours have fallen into a pattern of going dramatically downhill for several weeks once a year. This year and last there have been meds changes. I can’t remember whether there were the year before but will look into it. How any placement can meet her needs when she's in this state, I don't know. They can only contain her. However, this hospital placement has been promising far more than it delivers for months now & the behaviours have only recently escalated. I don’t attribute them to the environment, which will have remained largely unchanged. As I've said, the Topiramate reduction is likely to be a factor, if not the cause of her unsettled behaviour. However, I would still like her behaviour charts examined to see when she became more unsettled, as a thyroid medication was introduced on 20 September, which correlates with the behaviour changes. I’m told that it’s a tiny dose & wouldn’t cause this sort of problem but, with Chrissy, I always make extra checks as she has such paradoxical & unusual reactions to medications, especially when they are first introduced or changed.

So on Tuesday, Chrissy was at home with my mum and I. It started off ok but, when I was holding her hand, I noticed it was twitching & shaking. She grew increasingly demanding & obsessive about when dinner was ready, what’s for dinner, the computer, DVDs etc. She made her usual ineffectual attempts at spitting & hurting me with slaps on the shoulder. Her obsessions led to three sessions of prolonged, impulsive/compulsive SIB. They were so violent & prolonged I rang the hospital for help – something I’ve never done before. Mum said she hadn’t seen anything this intense for ages, & observed how Chrissy seemed ‘possessed’ by what was happening to her, & wanted to stop but couldn’t.

When Chrissy had recovered she suddenly beamed at me but, even in between outbursts, the smiles were short-lived. She was generally more agitated & demanding than usual. At around 9, Chrissy let me bath her and put her to bed. She then sat silently on the toilet seat in the dark for about 20 minutes, & called me when she was ready to move on. If you intervene before she's ready she will kick off again. She needs to be left alone. I call these her ‘frozen states’ and they often occur between violent SIB. Mum & I were struck by how changeable Chrissy was overall. At one point we asked her what had been the matter; she smiled sweetly & said ‘I had a tantrum’ but wouldn’t be drawn further.

That night, she slept right through until 2pm the following day. We managed to avert any major crises until the car journey home where she became agitated and obsessive again, & kept undoing her seatbelt & trying to climb into the back. The hospital reported that there were further ‘behaviours’ later on and the following day. I also learned that they, too, had seen them occur without any environmental trigger. I was told that while we were away, she had even got out of bed at night, run into other patients' rooms & jumped on top of them - to occupy their beds rather than hurt them. This barging into other people’s rooms is something she's been doing at home. Chrissy then threw & smashed someone’s tv set & radio – first I’d heard of any of this. These problems at night are exactly what happened in her residential placement in West Sussex at the end of 2010. When she’s settled she normally sleeps well.

The compulsive/impulsive nature of Chrissy’s self-harming behaviours has never been examined. One of her psychiatrists put it down to ‘autistic crisis’ and another to ‘attention-seeking!’ Her psychologist put it down to 'jealousy' of another challenging patient! We've never seen any evidence that Chrissy experiences jealousy as such - I can only think it's an unfortunate choice of words. English isn't her doctors' first language. Her previous neurologist wanted her to see a specialist in movement disorders and tics. We’d asked for the referral to go ahead when she was admitted to hospital but, at the time, her psychiatrist felt that the Maudsley (who came out & assessed her) would be able to offer everything she needed. She also said that the medications Chrissy was on would be the ones used for tic or movement disorders.

We can only hope that Chrissy will emerge from this period very soon & that we'll eventually help her to achieve lasting stability again as she did during the last six years of her teens.

A #Specialsaturday Post - Uncertain Times & Embarrassing Moments

The meeting we had yesterday to plan Chrissy’s future care was chaotic & disheartening. There was a roll call of apologies for absence – people worked on different days, a ward ‘crisis,’ someone away on a training etc. One of Chrissy’s care managers was present but he has no influence as his authority are not paying for her care. No one from the commissioning authority (funding ‘without prejudice’) attended. The psychiatrist has been unable to get hold of anyone there, which means we can’t plan Chrissy’s future.

I’ve already blogged about the funding dispute that’s leaving Chrissy in limbo. It will take a long time to plan her next placement as we need to get it right this time. A robust healthcare support infrastructure needs to be in place for starters. Another placement breakdown would be disastrous for Chrissy. The appalling state she was in through the latter part of 2009 still haunts me.

The hospital admission in January 2010 has improved Chrissy’s life. She is far more stable than she was 18 months ago but these changes have plateaued over recent months and we fear there will be little or no further improvement because the institutionalised environment isn’t geared up for her autism. There aren’t enough stimulating accessible activities offered. For months we were promised sensory integration therapy (‘The missing piece of the jigsaw,’ Chrissy’s speech therapist had said in March). Yesterday we learned that there had been a U-turn – they can’t supply it as part of the package; extra funding will have to come from the commissioners - the ones they can’t get hold of!

The meds changes took months to start due to all sorts of reasons (including the funding dispute) but we can’t thank Chrissy’s neurologist & psychiatrist enough for flagging up their concerns about one of her anti-epileptic drugs, Topiramate. We marvel over how much she’s ‘woken up’ since it was reduced from 50mg a day to 15mg. She is far more aware of her surroundings, and full of quirky observations & funny comments again. Last night, I was having eyelash extensions done for a special occasion. Chrissy was in the same room, playing quietly on the computer under Ian's watchful eye. Suddenly, she sat bolt upright, & announced: 'I poo myself in the kitchen.' The lady doing the eyelash extensions froze then we both collapsed into giggles as I explained that Chrissy probably felt the urge to go but had her tenses mixed up. There have been many potentially embarrassing moments like this but we just find them endearing, entertaining & funny. It's a good sign as it shows her irrepressible personality is no longer being clouded by too much - or the wrong - medication.

Embarrassing moments are part of what makes Chrissy who she is. We welcome them back!

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#Specialsaturday post - A Spooky Coincidence

Because I’d had a microdiscectomy on Monday, I wondered if it was wise to have Chrissy home this weekend. After learning from her nurses today that she was relatively settled, I decided to give it a go. My younger daughter, Alex, would be here until their step-dad, Ian, came home, so if things kicked off... What I hadn’t bargained for was things kicking off immediately Chrissy arrived. As soon as her nurses brought her home this afternoon, she started asking for dinner - then repeating obsessively that she wanted chips. It escalated too quickly for us to prevent her from taking to the ground. For the 2nd time in weeks she stripped off on our front drive & had a meltdown - the most violent I've seen since Xmas. Her nurses said they'd seen meltdowns like this on the ward since her Topiramate had been reduced. They’d obviously passed us by until now. Spookily, Chrissy had a horrific looking abrasion on her back, exactly where mine is from surgery. As she scraped herself backwards along the gravel on our drive, stones & dirt stuck to the wound. Chrissy continued to thrash about, oblivious to the pain. I marvelled over how she’d never suffered a serious wound infection. The high-intensity outburst must have lasted about an hour in total and I realised, with a sinking heart, that it would be far too ambitious for me to have her home in such an unpredictable state while I was still recovering from surgery. The three of us shielded Chrissy's nakedness from the busy road outside with a blanket & her coat, & waited it out. It faltered for seconds then started full force again complete with foot-biting, hair-pulling, skin pinching & finger twisting. If we got too near we got kicked & hit out at too. “Go away!!!” she shrieked. A painter had been working at the back of our house & had now finished. I asked Alex to tell him to wait before he came round the front to his van. He could hear the ear-splitting screams clearly. "It's my sister - she's autistic," Alex explained. What else could she say? Finally, for no obvious reason, Chrissy stopped, got up & was immediately docile & compliant, chatting away as if nothing had happened. She let us dress her over her dirt-covered body & take her inside to wash her hands. She then hovered excitedly as I packed up some food for her to take back with her. "What was all that about?" Alex asked. "Had a tantrum," Chrissy said matter-of-factly. I then settled her back in the car. As she smiled sweetly at me through the car window, I felt like I'd let her down, & had a little weep in Alex's arms. Later, her nurse called to say that Chrissy was fine, & had remained settled. She also reminded me that, because Chrissy's concept of time is different to ours, she hadn't realised that her home visit had ended before it had begun. Chrissy was just happy that she'd been home to see mummy. This blog post is part of the awareness raising campaign - Special Saturday - raising awareness of people living with special needs around the globe. 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://specialsaturday.org/home/"> http://www.facebook.com/SpecialSaturday