Being Chrissy's mum

A beautiful day...

But behind the scenes, I'm struggling with feelings of sadness and loss. We were so thrilled to get Chrissy back at the end of last year - to see the return of her zest for life and her enthusiasm for doing jigsaw puzzles, to take her on holiday, out with family, and shopping for new shoes, her grandma's reaction when we took her up north to visit her for the first time in years.....

Now, Chrissy's slipped away from us again and I'm gripped by the green-eyed monster whenever I see or hear about other adults with learning disabilities going out with their parents. It's routine for many such families but for us, outings and holidays with Chrissy are rare and precious events, often fraught with drama, to be celebrated when they go well. Chrissy just can't manage them at the moment and I realise that we're running out of treatment options. Her epilepsy no longer responds to monotherapy and nearly every secondary antiepileptic drug out there has been tried. She is so acutely sensitive to the neurological mayhem these drugs can cause that they stop her living her life. She isn't complying with bathing, teeth-cleaning etc and is still refusing food. Mood swings continue to be marked - one minute smiling and calm, the next screaming and shouting. She is disrobing on the unit but we haven't seen this at home - just shoes and socks off. She has bruises all over her body again from self-injurious behaviour - the only way she can express her distress. Two weeks ago I'd noticed bruising on her neck & had to raise my concerns at a higher level. I've never seen bruising there before & we're now awaiting the outcome of further investigations into how & when it happened. The explanation I got was too vague for comfort.

The weekend before last when that picture was taken Chrissy was so excited to see her brother, Jamie. His girlfriend, Penny, looks a bit like her sister, Alex. Chrissy thought she WAS Alex - & not just fleetingly. When Alex herself turned up later, Jamie and Penny had left. Chrissy transferred the name seamlessly to the real Alex. I'm uneasy about her confusion. She's always known who close family are even if she forgets other people's names. Another point to mention to her neurologist on Thursday when her antiepileptics are reviewed. The deterioration in Chrissy's health & mental state is something that he will be trying to resolve. The unit has no epilepsy specialists - the neurologist is based at a local NHS hospital. There's nothing more the unit can do for her now and, given the current situation, it's detrimental for her to stay there. So why is she still there?

Some of the housing barriers we've faced have shifted but new ones have popped up. We made a successful bid on a council house in the village but, although we were never given the chance to view it inside, we went with the professionals' views that it was unsuitable due to a number of safety issues in relation to where it was situated and the internal layout. The more housing options we consider the clearer we are about non-negotiables but it's hard to keep having your hopes raised only to have them dashed again. There are very few houses that become available in this area but Chrissy still has priority banding so I will keep checking the social housing website. The other option that's starting to look promising is shared ownership. A specialist housing association has finally come on board and we're having discussions about a house for sale nearby that meets their budgetary requirements. They are concerned about the noise impact on neighbours from any house with a party wall, even with sound-proofing, but a detached property is out of the question due to cost. The noise/disruption to neighbours issue must be a common theme with most people being moved out of assessment and treatment units into supported living and the Winterbourne View Joint Improvement Programme needs to address this.

The tendering process for providing the care package is also moving painfully slowly. I know we'll get there eventually but Chrissy has now spent 3 1/2 years in a locked ward. "What must it be like for her?" I asked Ian recently in a tearful moment. "We'll never know," he said. "You try to put yourself in her shoes but you can't see it like she does and there's no point even speculating."

He's right of course and I need to get a grip - but what mum wouldn't feel like I do? My heart is always at war with my head. I long to kidnap Chrissy and bring her home but I know the situation would be unmanageable, and certainly not in her best interests.

Last week Alex asked: "Do you ever resent Chrissy?"

"No," I answered as honestly as I could. "None of this is her fault. It's the situation I resent."

Reflecting on this question today I can recall times when I've felt fiercely resentful, but only in passing, the same way that I resent Ian when he keeps does something infuriating no matter how many times I ask him not to - like dumping his work briefcase on the kitchen floor!

Alex has just started work as a tutor at a unique school for children with autism, using ABA (Applied Behaviour Analysis) http://www.ambitiousaboutautism.org.uk/page/treehouse_school/teachingandlearning/aba.cfm She's very excited about this new opportunity. She has always felt helpless in the face of Chrissy's suffering, even as a small child. Now she feels able to channel her conflicting emotions, and to use her valuable family experiences and training as a psychologist and teacher to improve the lives of other young people who struggle with autism and learning disabilities. Perhaps we can, in consultation with Chrissy's support team, try some ABA strategies once she moves into supported living.

Naively perhaps, I'd once hoped that similar autism-specific approaches would be used in assessment & treatment units & residential homes that claim to specialise in autism. Maybe one day.....



I support Unique http://www.rarechromo.co.uk/html/home.asp & I'm a SWAN (syndromes without a name) blogger

Living with a Rare Chromosome Disorder (2)

Just as I was brimming over with joy about Chrissy getting her life back, it all went belly-up again- but every cloud has a silver lining. Reading through Chrissy’s records and seeing everything that’s happened over the past six years was harrowing but now I'm clearer about what she needs for a better future. Her recent setback is a stark reminder of her fragile hold on stability and the importance of having robust community-based medical support.

Chrissy’s behaviour started to worsen after her antiepileptic, Zonisamide, was in the final stage of being titrated up to 300mg a day. Her moods became very erratic and she turned into a roaring Stalinesque dictator. Then she kept going back to bed and lost her appetite completely. As Chrissy is obsessed with food and eats everything that’s put in front of her, (the opposite of her failure-to-thrive infancy) alarm bells rang.

These symptoms had appeared nine years ago when she was 20 - her platelet count had dropped so low she was rushed to hospital. So this time, I asked for blood tests to be done asap. Lo and behold, her platelets were low again. Platelets help the blood to clot so if the count drops too low it can lead to uncontrolled bleeding. There have been other abnormalities in Chrissy’s blood count over the years, nothing too striking, but at one point her doctors thought she had myelodysplasia, or pre-leukaemia.

So here we go again. This time, her platelet count isn’t low enough to put her at risk but it’s been part of a general downward trend. As any parent knows, there’s nothing worse than seeing your child suffer but knowing you’re helpless to do anything about it. Last weekend Chrissy kept telling me - ‘I feel sick.’ Sometimes she tried to eat but couldn’t bring herself to swallow; other times she refused food and fluids altogether. She has lost 7.5 kg (over a stone) in the past fortnight and constantly has white gunk around her mouth due to dehydration. Her doctors have been on the case but it’s trial and error. Chrissy’s neurologist advised a reduction in Epilim - not the new drug, Zonisamide. I agree with his decision. So many new adjunctive antiepileptics had been tried, and caused horrible side-effects, we were at the end of the road. We can’t keep putting Chrissy through what are effectively drug trials. She’s been on Epilim for years so it’s a case of better the devil we know, but the dose has been high, and, perhaps when Zonisamide was added it tipped her over the edge. We saw Chrissy’s neuro on Friday and he said that Chrissy had been trialled on just about every antiepileptic drug going, so he was reluctant to try any more. He decided to withdrew Clobazam, as that could be making Chrissy nauseous, and should have only been a stop-gap drug while Zonisamide was being titrated up anyway. If Chrissy responds well he will look at tweaking her other anti-epileptic drugs down to try and achieve a better seizure control/quality of life balance.

The sudden deterioration in Chrissy's health has been a rude awakening. I’d been living in hope that one day she would regain the lasting stability she enjoyed during her teens. Now I have to admit it’s unlikely. It was hard enough to stabilise her epilepsy and behaviour but now these other side-effects have to be taken into consideration too.

So until a few days ago, I was going through a period of grief again - bursting into tears at unexpected times, and waking up in the night panicking, and torturing myself with thoughts like ‘how can her body cope with all those drugs going into an empty stomach?’ Then the sun came out, and love and support came pouring in from all sides, and the future started to look rosier again....

In the meantime, a bungalow has come up on the social housing register. It looks promising – in a neighbouring village set in open countryside, five minutes drive from us - and Chrissy would share our GP, who already knows about her. Her Social Worker is 'quietly confident.' We should know by Monday if our bid's been successful.

Supported living still feels like an experiment to me but I'm told it can work for even the most complex and challenging individuals. If it's going to work for Chrissy, it stands a better chance if she's close to her family.

Wish us luck!



I support Unique http://www.rarechromo.co.uk/html/home.asp & I'm a SWAN (syndromes without a name) blogger

Living with a Rare Chromosome Disorder

Well....Chrissy's clinicians have deemed her ready for discharge now, just as I was beginning to wonder whether I was kidding myself to think that her behaviour and epilepsy would ever be stable again.

Since the last meds change several weeks ago, every day has been a sunshine day. We're almost at the optimum therapeutic dose of Zonisamide, at which point the stop-gap antiepileptic, Clobazam, will gradually be withdrawn.

It's heartwarming to see 'no seizures' and 'no challenging behaviours' recorded each day in Chrissy's ward/home report. The episodes of inconsolable distress have vanished. Music from her Ipad makes her dance in her chair and her cheeky chuckles ring out around the house. I took the photo above when we were sitting in my car and she had just reached across to stroke my arm lovingly. Simple gestures like that mean the world to me as Chrissy doesn't often instigate affection.

She is having a great time exploring the new ipad she got for her 29th birthday, doing puzzles, building snowmen and causing mayhem in her virtual home. At first she used different fingers and even changed hands to manipulate objects around the screen - but she's learning fast. She has relatively good manual dexterity and loves anything to do with computers.

On our morning walk to the village shop, Chrissy pointed out sights that interested her, including a metal arch over someone’s gate. 'Rainbow!' she cooed, then stood admiring it for a while. I was enchanted.

Chrissy admiring the 'rainbow'
Chrissy still struggles with transitions and needs all our attention but she's content and responsive, which makes our job much more rewarding. She wants to be out there living her life now. The unit do their best, and do take her out, but not as often as she would like. This morning when I asked her to take her medication, she replied: 'When the night staff come!' It's definitely time for her to move on but we've had to involve solicitors to focus commissioners' minds as things were dragging on. This has been the case with each of Chrissy's major transitions over the past few years. Legal aid cuts are brutal but, in this instance, Chrissy's solicitors were able to demonstrate that we had exhausted every other avenue and only turned to them as a last resort. She will, however, have to pay a contribution.

Chrissy's housing options are still unclear but we are finding a way through the maze. One of the biggest barriers we faced was accessing social housing. I was advised to bid on local homes by Chrissy's commissioners but after several bizarre 'admin errors,' a housing officer said that Chrissy wasn't 'well-placed' for general purpose social housing as she was 'unlikely to be thrown out onto the street.' If someone without a learning disability is at the point of discharge from hospital they would be moved to a priority banding..... The very few adapted properties that I've seen on the website are for the elderly only and, although Chrissy moves slowly and cautiously, she doesn't need adaptations for limited mobility. I then learned that there was a supported living panel at our local CC, which we had not been told about. Our local council's policy documents state that social housing for vulnerable adults and those with a learning disability are their priority. This hasn't been our experience and we've put in a Freedom of Information request to identify how many people with a learning disability have been offered social housing in the CC's area over the past year.

We've also made a mortgage application on Chrissy's behalf, which would be paid for from her benefits, and we're still exploring shared ownership schemes through registered social landlords. I had been left to deal with all this confusing information but now I've had enough and forward emails to the commissioners to deal with.

Chrissy finally has a social worker! Perhaps the long-awaited community care assessment will be done now. She must surely benefit from Social Services and NHS commissioners working together but why has it taken so long when the planning process has already taken place?

So, I'm still fighting to get Chrissy an appropriate care and housing package in the community, but the sting has gone out of it because she is so settled and content. This stability was only achieved by her spending time in an assessment and treatment unit and, in our view, the Government's knee-jerk reaction after Winterbourne to close down such units is misguided and dangerous. There's no doubt that big changes needed to be made, not least for assessment and treatment units to live up to their name - to assess and treat their patients - but we have much to thank Chrissy's unit for. They've brought her back to us.

I support Unique http://www.rarechromo.co.uk/html/home.asp & I'm a SWAN blogger

A Special Celebration & Undiagnosed Children's Day

When Chrissy arrived during the hottest April for 100 years, I was the proudest mum in the world. I was 23 and a first-time mum. It felt like my greatest achievement ever.

My baby looked flawless and her beautiful dark eyes seemed to take in every detail of her surroundings. In her little perspex cot on the maternity ward she waved her hands around so energetically that I called her our 'air traffic controller.'

Then the dark shadows started pressing in. Thank God we can't see what lies ahead of us.....

Like many children with undiagnosed genetic conditions, Chrissy was a failure to thrive baby. She struggled to feed and gain weight, and was floppy and sleepy - but she was a mass of contradictions. Mostly she was sunny-natured, alert and content to coo and watch the world go by but, even in infancy, she had prolonged spells of inconsolable screaming, and no one knew why. The rest is history and detailed in my book: 'Bringing Up a Challenging Child.... http://www.jkp.com/catalogue/book/9781853028748

Although we now know the cause of Chrissy's problems, she still remains quite an enigma to us and to her clinicians, despite spending three years in an assessment and treatment unit. We know her chromosome disorder causes hugely variable effects but her learning disabilities are significantly worse than most people affected by 1q21.1 microdeletion, even those with a larger area of deleted genes.

So where are we 29 years on?

We have another CPA (care plan approach meeting) coming up on Monday that we never expected to have. Chrissy has now been in the unit for over three years and is ready for discharge but despite my monumental efforts to find solutions and move things forward, she is still stuck there. The Government is very clear that people with a learning disability should be supported to live in their own communities so it is difficult to understand why there are so many barriers in place. I feel like I'm stuck too. It has been left to me to drive it all, and I now spend most of my time on this. It's the same with every major transition, eg when she went into the unit I was driven to the brink of despair along with her. Not only was I in pieces over her suffering but I fought epic battles to get her into hospital. Why does it have to be like this?

Mencap are very interested in Chrissy, and are finding it hard to understand why the situation has dragged on for so long. They are incredulous about the amount of correspondence that I've had to engage in to try and understand the barriers, and to try to move things forward for Chrissy. Her case has been brought to the attention of Chris Bull MP, who is leading the Joint Improvement Programme to move people out of assessment and treatment units after Winterbourne, and Norman Lamb MP, Minister for Care, who has asked to be kept informed of progress relating to Chrissy's case.

Our experiences are echoed by this excellent article highlighting flaws in the Government's plans to move people with learning disabilities and challenging behaviour out of assessment & treatment units into community-based settings:
http://www.communitycare.co.uk/articles/10/04/2013/119078/dont-ignore-housing-in-response-to-winterbourne.htm

Chrissy's most recent (and hopefully last for now) medication change has been from the antiepileptic, Keppra, to Zonisamide. Because she had three seizures in a row after Keppra was withdrawn, Clobazam, a benzodiazepine derivative like Valium, was added to Zonisamide and Epilim. Clobazam will be withdrawn when Zonisamide is titrated up to the therapeutic dose. Chrissy is already on the maximum therapeutic dose of Epilim. I've discussed seizure control/quality of life and my fears about the effects of medication on Chrissy's mood, health and general well-being in previous posts. Clobazam's sedative effect is dragging Chrissy down. I can't wait for it to end as it breaks my heart to see another drug stealing away her personality, and sapping her energy and intellect. I have to remind myself how far she's come - she rarely has screaming outbursts now and, as the effects of her medication wear off during the day, Chrissy is her old self again. It's a delight to see her enjoying jigsaw puzzles again, a skill she excelled at as a child.



Now Chrissy is more aware, she says with increasing frequency: 'I don't want to go back to the hospital.' I have sleepless nights, guilt trips and anxiety attacks over how she must feel living in a locked ward for so long, her suffering and disorientation from the effects of different medications, what she's gone through over the years, what the future holds for her etc. Of course I can only imagine how I'd feel in her situation but, no matter how hard I try to banish those thoughts, they haunt me. It would be such a relief to see her settled again near her family where we can be more involved on a daily basis and help with her support. It's the least she deserves.

I support Unique http://www.rarechromo.co.uk/html/home.asp & I'm a SWAN blogger

Happy Times!

Ian was away on business so it was just Chrissy & I this weekend. From the moment I picked her up she was jolly and chatty. Throughout her visit there were fewer shouting episodes and, unlike last week, no swearing.

Overall, Chrissy was more compliant and able to focus on the task in hand. This morning I let her take things at her own pace. She chose to play on her laptop before doing anything else. She then let me know when she was ready for her bath and, after I got her dressed, I left her to eat her breakfast on her own with no distractions. Her obsession with food vanished when she came off antipsychotics - but surprisingly she hasn't lost weight. Hopefully we will be able to address her diet when she is living in the community and no longer eating communally.

I was even able to have a shower while Chrissy played quietly downstairs on her laptop. Unheard of! I sneaked downstairs to check that she wasn't stalking one of our cats and was reassured to see that she was sitting contentedly at the kitchen table selecting a DVD. This ability to entertain herself is new - usually, if she is left alone in a room, she seeks us out and shadows us so closely that we get stuck in doorways and fall over her whenever we turn round. She has taken up old hobbies like drawing and writing again.

'My Dinner' by Chrissy

On our morning stroll to the village shop Chrissy clutched her banana-&-chocolate-mousse-in-a-bag and posted my letters then stopped for chats with a lady that used to work behind the till and my neighbour. It reinforced my view that she should move into a familiar community where she will be among people that know her.


As I drove her back to the assessment & treatment unit she kept smiling at me and stroking my shoulder. These genuine shows of affection have been increasing over the past few months. She often spontaneously kisses Ian and I, and says: 'I love you sometimes.'

Only downside is that she's had two nocturnal seizures this week but one or two a week is fine and may account for her calmer behaviour.

Tomorrow Chrissy starts on vitamin B6 (pyridoxine), which may curtail some of the negative behavioural effects that we've seen since she's been on Keppra. I was pleased that her psychiatrist took it upon himself to do his own research & agree to a trial of vitamin B6. Chrissy's neurologist hadn't been convinced.

So this weekend Chrissy's behaviour, ability to engage, alertness, focus etc. were as good as they were just before she went on Keppra but was having too many seizures. Have we finally achieved the successful balance we've been seeking for so long?

Only time will tell....




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

Epilepsy & Behaviour (2)

I first posted on the reciprocal relationship between epilepsy & behaviour a year ago http://jgregorysharingsstories.blogspot.co.uk/2012/01/epilepsy-behaviour.html This post is on the same topic with a different drug.

At the end of last year I told how we'd got Chrissy back at last. Unfortunately her seizures took over and, just before Christmas, Keppra was introduced as an adjunctive antiepileptic to Epilim. Since then perfect seizure control has been achieved but her behaviour has become increasingly bizarre. Since Keppra's most serious adverse effects are behavioural, we need to closely monitor its benefit-risk ratio for Chrissy.

Ten days ago I agreed with Chrissy's doctors that we should halve the dose to try to achieve a better seizure/behaviour balance. Last week I thought things had levelled out when her ward manager said that she seemed much more herself. Trouble is he'd only called in to see her in passing, & appears to have reported a snapshot view. Other ward staff have reported similar behaviours to those we've seen - shouting, swearing,& inability to focus. Yesterday her nurse told me that she was too distracted to take her morning meds - although they somehow got her to take them eventually.

At the weekend we, too, were unable to get Chrissy to focus on the task in hand. We were dismayed to see how bad things had got. She had periods of being herself then suddenly exploding into staccato roars & shrieks: 'Mummy! Shut up! Bastard shit! Fucking bitch! I spit at you!' It's like someone with Tourette's Syndrome on overdrive. She either stalked us as if she was trying to goad us or took herself off to a chair on the landing, still shouting. These shouting episodes can last for an hour or more, and even when she settles she's still very restless and whingey.

On Saturday morning I had a struggle to get her to focus long enough to climb into the bath, take her tablets or eat her breakfast. She kept up a constant stream-of-consciousness commentary as if she was on speed. She eventually exhausted herself, & my patience. At one point she said: 'Chrissy's poorly & screaming.' (In fact we haven't seen any screaming, stripping behaviours.) 'Poor Chrissy,' I said, & signed: 'Are you cross or sad?' As she answered & signed 'sad' my heart melted & my irritation with her evaporated.

Eventually she sat cross-legged on her bed & said: 'Who's that? What's that name?' I followed her gaze to the foot of her bed. Did the pattern in the rails look like a face to her? 'There's no one here,' I said. 'Is it a witch?' she asked. (A new word she uses frequently but not always appropriately!) She leaned forward & pressed a gold circle in the pattern that looked like a button. 'It doesn't work,' she observed. Then she started shaking the curved rails at the end of her bed as if she expected to bring them to life!

Since all the medication changes Chrissy has been far more aware of everything around her but this was very odd. She then asked me to 'do' her covers, burrowed into them & said 'goodnight mummy.' I left the room & when I came back 10 minutes later she was fast asleep. It's unheard of for Chrissy to get into bed fully clothed without asking for her curtains to be closed, whatever the time of day.

All these changes in Chrissy's behaviour & normal routines make me feel very uneasy. It's as if her personality is being sabotaged.

I had first discussed the timing of the dose with her ward manager 11 days ago. We'd agreed that it made sense for the dose to be given in the evening, as most of her seizures are nocturnal. However, for some reason Chrissy's doctor had prescribed it in the morning. I am still waiting for him to review the prescription. I had also passed a message on to Chrissy's doctor to ask him to consider adding vitamin B6 as studies have shown vitamin B6 deficiency may occur with some patients on Keppra, and that its addition can curtail some of the adverse psychiatric effects. I'm still waiting for answers & hoping that this is a temporary setback that we can find a way through - otherwise we're on the roller-coaster ride of yet another drug trial.



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

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

Enjoying life: Chrissy in 2003 with her sister Alex

After years of suffering, your child is given a 'wonder drug' that transforms her life - then it is snatched away because it could kill her. Over the next seven years different alternatives are tried but nothing else helps. What do you do? Do you try it again, under close medical supervision and make the most of the quality time it gives your child - or do you dismiss it as too risky?

This is what happened to Chrissy with Naltrexone.

In previous posts I've spoken in more detail about how low-dose Naltrexone stopped Chrissy's self-injurious outbursts, and why it was discontinued. The following information from Autism Research Institute explains the physiology. It's important to not that when self-injury is associated with a biochemical abnormality, there may be little or no relationship between the person's physical/social environment and self-injury. Thus, the behaviour may occur in various settings and around different people but may occur less frequently during situations in which the person's behaviour is incompatible with self-injury, such as eating, playing, and working on a task.

Here, I'm more interested in exploring our dilemma over Naltrexone, which has divided opinion between some of Chrissy's medical specialists and I. Those that have done their research and/or seen its effects consider it to be a very safe drug but, some of them are wary prescribing it off-label. Naltrexone isn't a drug that is closely linked to thrombocytopenia but Chrissy has a history of rare and paradoxical side-effects from medication.

She was on three different drugs when thrombocytopenia was diagnosed, one of which was Epilim, an antiepileptic drug that she's been on since childhood that has proved the most effective at controlling her seizures with minimal side-effects. Naltrexone and Prozac were introduced together during drug trials when Chrissy was 14 in an attempt to quell unexplained self-injurious outbursts that were escalating in frequency and severity. The combination of the two was unusual and I don't know where the suggestion originated. Seven years of behavioural stability followed during which Chrissy was able to go on holiday abroad and enjoy activities in the community. Then when she was 21 Naltrexone and Prozac were withdrawn together because by the time her thrombocytopenia was detected it was a medical emergency. Her platelets reverted to normal levels within 48 hours so her doctors concluded that the thrombocytopenia was caused by a combination of Prozac and Naltrexone. I have

Epilim was continued but the dose was halved about a year later when Chrissy's platelets dropped slightly again. This shows that at higher doses Epilim alone affected platelet levels.

Three years later Prozac was re-introduced, albeit at half the previous dose. There was an improvement - not life-changing but enough to work out that if Chrissy wasn't taking it things would be even worse. There have been no further concerns over her blood count, which is checked routinely.

So Chrissy is now on two of the three drugs, albeit at lower doses, that she was taking during her thrombocytopenia episode. Do we assume then that Naltrexone alone was the culprit or was it a cumulative effect of all three drugs?

The quality of life question came up last week. Framing it was the fact that Chrissy had enjoyed seven good years on Naltrexone. Now her blood counts are fine do we risk re-introducing it under close medical supervision to see if she can enjoy another long period of relative stability? Of course there are finer points to consider, ie, could Prozac be swapped with an alternative anti-depressant; is there an alternative opiate-blocking treatment to Naltrexone? Both these options would mean more drug trials......


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