One Month Before Heartbreak: From a mother’s perspective
Today, I am leaving the tabloids by the wayside in order to make a contribution to the One Month Before Heartbreak blog swarm, an event organised by The Broken of Britain and others to raise awareness of the government’s public consultation on reform of Disability Living Allowance (click if you would like to share your concerns with the government) which ends on 14th February 2011.
My family are fortunate enough right now not to be affected by whatever changes are finally decided on, but this was not always the case. The post below was first published on Ciao’s Café section in September 2003, and I hope it will help to increase understanding of some of the challenges facing disabled people, and their carers. I was intending to write a new post for this occasion, but I simply couldn’t find the words to express what I wanted to say. I hope you’ll all forgive me for this bit of recycling. This is the story of my daughter, Courtney.
***Courtney was born on 27 August 1992. I was the ripe old age of nineteen at the time! She weighed 6lbs 11oz, and was perfect. I did the usual check of fingers and toes – all there. The paediatrician at the hospital checked her over and gave her the all clear, and off we went home. Things all seemed fine at first – watching her grow was a pleasure, seeing how much she learned a joy.
Then I began to notice things weren’t quite right. Her speech was laboured, she was clumsy, she constantly leaned on the furniture to get around and she could not run or kick a ball. She could read the alphabet, name lots of colours and shapes, and was very good at counting, so these other things seemed odd to me. Whenever I raised any concerns with people, they would say ‘Don’t worry, she’ll catch up’ Being my first child, I wasn’t really sure what she should be able to do, so foolishly I ignored the little voice in my head that told me something was wrong.
Courtney had a health review at the age of two. This was carried out at home, and the health visitor concerned was not willing to listen to me. All she seemed to care about was finding out whether Courtney could name some pictures in a book – the fact that she could read the alphabet seemed to be of no importance. Of course, due to the struggle she had finding and pronouncing words Courtney was deemed ‘backwards – she’ll never catch up with other children’ (an opinion I rate very unhelpful), and the delightful health visitor left with a vague mention of speech therapy and was never seen again.
In 1996 at the age of three and a half, following a review with a much better health visitor and a visit to a consultant paediatric neurologist, Courtney was diagnosed with non-specific leukodystrophy, a progressive genetic disease where the white matter in the brain is destroyed by a faulty metabolism. This followed several blind alleys and incorrect diagnoses, and turned out to be the rarest of the rare. There are quite a few named leukodystrophies, but hers wasn’t one of them, hence the ‘non-specific’. Parents are often dismissed as needless worriers, but usually their instincts are right. At the time of diagnosis, she was given six months to live, but she actually managed another three years.
At this point, I would like to offer some advice regarding Disability Living Allowance. If you ever find yourself in the position of having to fill in one of the long and unforgiving claim forms for this benefit, make sure you spell out all special needs clearly on it. I say this because my original claim on Courtneys’ behalf was turned down as she was ‘not disabled enough’, even though she needed significantly more care than an average child of her age. When I first filled in the form, she had a tentative diagnosis of cerebral palsy. I received the letter regarding the unsuccessful claim on the same day I was told what was actually wrong with her – talk about insult to injury! Once a person has a diagnosis which means their life expectancy is six months or less, they will be entitled to the higher rate of DLA care component without a three month qualifying period, so this decision was overturned on appeal in Courtneys case (for useful websites, see below).
So, now we had a diagnosis what could we expect? The list of symptoms was long and frightening – increasing dementia, loss of vision, loss of hearing, loss of speech and ability to swallow, fits, incontinence – need I go on? The grief began immediately. I was to lose the child I thought I had, gain a much more demanding child, and then lose her too. I thought I would be unable to bear it, but strangely you do. It wouldn’t have been right to cry all the time in front of Courtney, so somehow we smiled, making inappropriate jokes to see us through the dark times. If you have ever wondered why the medical profession seem to have such a morbid sense of humour this is probably the reason. I was often told that I was brave, but that’s not true – I was just being a mother.
Following the diagnosis, Courtneys consultant referred us to the Make a Wish foundation. This is a charity which exists to grant the wishes of children with terminal or life-threatening illnesses. We ended up going on a long weekend at Disneyland Paris due to Courtneys’ love of The Lion King. As luck would have it, the weekend we were due to go the Channel Tunnel was closed due to a fire – instead of a two hour journey on the train we ended up travelling for twelve hours each way by car, train, bus and ferry. Ever felt like the fates are against you? It was even worse for me as I was two weeks away from giving birth! It was worth it though – I ended up with two great photo albums.
Following this, things didn’t go too badly for a while. With physiotherapy, speech and occupational therapy plus a place at a very good special school Courtney stayed on a fairly even keel. Her fits (grand and petit mal seizures) were controlled with Epilim (sodium valporate), Lamictal (lamotrigine) and diazepam. I found a support organisation called the Research Trust for Metabolic Diseases in Children (RTMDC now renamed CLIMB – web address below) who gave me a lot of useful information and were always available to talk to. As she was at school there was no need for any respite care.
It wasn’t until 1998 that Courtney had a significant deterioration in her condition. It began with laughing (jolastic) fits. These were caused by the disease entering the brain stem, the place where all the functions necessary for survival such as breathing, digestion and heart rate are located. The only drug available that could control these fits was phenobarbitone, an older drug which is quite a heavy handed barbituate. The most heartbreaking aspect of this was that we couldn’t even make Courtney laugh any more for fear of triggering a fit. When she was having one you could see how upsetting it was for her, and the fits would often last twenty four hours or more.
Then she began to have trouble swallowing. A naso-gastric tube was fitted in order to feed her, but despite being drugged up to the eyeballs and not having complete control of her body, Courtney managed to pull it out on several occasions. She never lost her cheekiness! The medical experts gave up on this idea, and decided to fit a gastrostomy tube instead. This goes directly into the stomach through the abdominal wall and there is a connection on the outside that enables you to attach any feeding equipment (feeding can be done manually or with a pump). The gastrostomy was a success, although I had to learn a new set of jobs including administering the special liquid diet and keeping what they call the stoma site (where the tube goes into the stomach) clean. It was a few months down the line before I was offered a pump, and feeding was very time-consuming until then.
An eye test then revealed that Courtneys sight was nearly gone. The disease had progressed to her optic nerve. Following this discovery there was a fairly rapid deterioration in her condition. Needless to say, she was no longer going to school. I had a lovely threatening phone call from the education department one day demanding to know why I wasn’t fulfilling my legal duty of sending her to school. I wasn’t very impressed, told the woman Courtney was dying, and advised her to read her records more carefully in future. She had the decency to apologise, at least.
By this time, I was finding it very hard to cope. For my twenty four hour a day seven days a week job, I was awarded the princely sum of £37 a week Invalid Carers Allowance (the amount may have gone up by now). I was also allowed two hours a week respite care, when a trained carer would come into the home so myself and my healthy child could get some time away from the house. This was not enough. I was cracking up. My relationship with my sons dad had come under so much strain that we split up. I told my health visitor that if someone didn’t help me soon I would walk out and leave the children behind (something that I continue to feel guilty about to this day). That same day, she referred me to Naomi House, a childrens hospice near Winchester which is entirely funded with public donations.
Off we went, not knowing what to expect. We were supposed to be staying for a weekend, but as it turned out we were there for three months. The resident doctor was unhappy with Courtneys medication, namely the phenobarbitone, so I agreed to let him attempt to cut the dose, which was originally why we ended up staying a bit longer.
What a wonderful place it turned out to be – not all doom and gloom as you might expect, but a place of laughter, life and fun. The fully qualified childrens nurses removed the burden of nursing so that I could be a mum again. It had been so long since I had enjoyed a simple cuddle with my little girl that I felt nervous doing it at first. The hospice has a suite of rooms for parents upstairs where the children aren’t allowed. There is a fully equipped kitchen, a living room, and four bedrooms similar to hotel rooms with en-suite bathrooms. This became my home. Every time Courtney was due to come home, there would be another set-back so we would stay for longer. Reducing Courtneys medication didn’t work, so it was put back up again. Eventually, she stopped digesting her food. She had to continue with a diet of water as this was all she could tolerate. A syringe driver (which automatically delivers drugs through a needle) was set up as her medication was not being digested. You know things are looking bad when diamorphine starts being used.
Courtney died on July 13 1999, shortly before her seventh birthday. Fortunately we were still at Naomi House. I was with her when she died, and it was very peaceful (what a cliché). It was almost as though she looked at me and smiled before she left. There is a special suite of rooms at Naomi House – one is a refrigerated room that is set up like a bedroom for the child, the other is like a sitting room (not refrigerated!) for the people they leave behind. I and my family spent a lot of time there, and also in the private garden reserved for such times. It is good, because you have plenty of time to say goodbye (though I did feel awful about leaving Courtney in such a cold room!).
A week later, we held a very personal funeral service for Courtney in what is known as the Dovecote at Naomi House. It is a circular room, and is similar to a small chapel. At the service I realised how many lives Courtney had touched. There were a lot of people there. Her consultant neurologist had come, despite being informed about it at very short notice. I left a memory book outside, and everybody wrote something funny, or sad, or special that they remembered about Courtney. It turned out to be quite a unique occasion, and there were some lovely readings. I had specified no mourning clothes, so it looked more like a wedding than a funeral, and instead of flowers people made donations to Naomi House – I think this raised over £300 in the end, a great tribute to a very special little girl.
So there you have it, C for Courtney. Looking back over it, I realise what an incredibly long op it is (and this is the edited version – there’s a lot I haven’t told you). If you have managed to read this far, thank you. Please don’t be too sad – Courtney would not have liked it. She was always very happy and would want you to be too. She came, she taught, she laughed, she left.
ps: I have given this a 5-star rating, but that relates to Courtney, not her disease which gets a big fat zero!
For information on benefits for the disabled and their carers – www.benefitsnow.co.uk
CLIMB (Children Living with Inherited Metabolic Disorders) – www.climb.org.uk
Make a Wish – www.make-a-wish.org.uk
Naomi House, the childrens hospice – www.naomihouse.org.uk
For information on epilepsy and seizures – www.epilepsy.org.uk
Jeans for Genes, raising money for research into cures for genetic disorders – www.jeansforgenes.com ***