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Saturday 16 Dec 2017
The Story of Ben PDF Print E-mail

Chapter 1

Ben’s Story

I have tried to write Ben's story to tell the reader about the problems and successes we have had in the hopes that it may be of support to others in similar circumstances. It is not intended for anyone who had a child with canavan disease to read this and think that their child will be the same as Ben, this story is his specific story.

I hope the moral of this story, that I would especially like to pass onto other families is that although there will be some really tough times, hang on in there, brighter days do come around.

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Particularly to families in the Uk I would like to say if there is something reasonable that you or your child really needs such as equipment or support then never ever take no for an answer, whoever it is that is telling you no. It's really hard but you have to make sure the problem is heard higher up, shout louder, don't give in, it might take a long time, much longer than it should, but there is always a way.

Try and gain the support of those professional people that you feel do understand your needs and don't allow those that don't to cause you additional stress. Always deal individually with the department concerned ie don't let your social worker tell you the rules of education, speak to education directly and so on.

I have tried to keep this version of Ben's story brief, to tell you how proud we are of Ben, how much we love him and how his diagnosis has broken our hearts would fill a large book.


IN THE BEGINNING

Ben is our first child, he was born in January 2003 in Norfolk, England. We had suffered two twin miscarriages before getting pregnant with Ben so already we knew how precious this gift of life was. After my miscarriages Adrian and I were tested to check if there were any problems for us to conceive a healthy pregnancy, the tests came back all clear. During my pregnancy I had my triple test which showed risk of spina bifida (this has nothing to do with canavan disease) so I had a scan every month to check the development of Ben's spine which was fine. I thought in a way I was quite lucky to be able to visit my baby via a scan so many times during my pregnancy and watch him grow and was filled with such pride and hope.

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Baby scan 11 weeks Soles of babies feet 20 weeks Babies face looking upwards 31 weeks

Ben arrived full term and after an 18 hour labour via a ventose delivery, he weight 5lbs 15oz, it’s common in our family to have small babies. Initially his Apgar score was 1 and he had to be resuscitated.

The hospital was very busy and we were left in the delivery room for over four hours after Ben was born. We were concerned about his general well being and asked several times if he was ok, we were told it was just the drugs used for labour. During this time not being moved or washed I developed a bed sore 11cms square.

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Special Care

When Ben and I were finally given a bed on a ward it was discovered that Ben had seriously low blood sugar of 0.1 and was hypothermic, he was rushed to special care. I slept for 4 hours having been awake for 40 then woke at midnight, a special care nurse had put a picture of Ben on my bed whilst I was asleep. I tried to find Ben and stumbled up and down the ward dragging a leg still numb from anaesthetic but could find no staff to let me out or back in.

Ben spent a week in special care, initially in a full incubator and later in the week a small cot. We were told that after his birth Ben had had a swollen brain like concussion causing him to have a seizure but this had gotten better. We were told that we would receive visual, hearing and paediatric follow up appointments but these were routine and nothing to worry about and that his vision was fine.

The special care baby unit advised us to use an apnoea alarm and loaned one to us.

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Going Home

On arriving home a week after his birth life wasn’t easy looking after baby Ben. He could not feed well, he would spend 40 minutes breast feeding and then still drink a full bottle of either expressed breast milk of formula.

He was increasingly difficult to settle and get to sleep.

One afternoon Ben was particularly poor at feeding, the midwife came for a routine visit and found that his temperature was dropping. We went back into hospital for the afternoon and evening, Ben was tested for infection but this was clear, he picked up and we were allowed home, there was no explanation.

In the coming weeks and months Adrian would have to stay up until 11-12pm each night feeding Ben to sleep and then I would take over as Ben woke several times a night for feeds or to be settled. Determined to breast feed I expressed breast milk for Ben but to keep the milk supply coming I had to express milk every time that Ben fed, including twice a night. This was despite our consistent effort to get Ben into a bedtime routine.

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Our concerns begin

Ben was also seen at home first by the midwife who delivered him and then by his health visitor, we were concerned that Ben did not smile but were told that babies that had been in special care could have their development set back by up to a month and that we should not be concerned.

Routine follow up appointments came and went, Ben gained weight steadily and it appeared that Ben was fine and making a full recovery after his birth. No professional told us that they were concerned about Ben and we didn’t understand the occasional bit of terminology on the copies of the clinic letters that did come to us.

Ben had increasing feeding difficulties, he cried when he was hungry then he cried when he was fed. He settled eventually when in physical contact with his parents but screamed and screamed when he was put down. He screamed if his parents tried to take him for a walk and he screamed in the car. He screamed anytime he was undressed be it for a nappy change or to have a bath. He would not eat unless he was in his own home and when out would just scream with hunger but refuse to drink.

[Dad says: In the evening Sally would go to bed early ready for the night time routine and I would stay up and put Ben to bed, It's no joke that I would sit with Ben is bedroom trying to get him to sleep whilst wearing industrial grade ear defenders, his crying was so persistent and loud. The ear defenders help increase the tolerance to the screaming which would last anything up to 2-3 hours].

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Ben fed every 2 hours from 5am until late at night. It took an hour to feed him and settle him down, then I had to express breast milk, get the breast pump washed up and back in the Milton tank by which time it was nearly time to start all over again. As time went on Ben started screaming in the early evening, even when being cuddled, we couldn’t sit down together after dinner, the only thing that stopped Ben crying was to walk him slowly round and around the house. This was despite trying all the professional advice we had on trying to settle him, he was in pain but we didn’t realise how much. We were exhausted.

At 8 weeks old Ben’s paediatrician gave us Gaviscon to try and help Ben’s feeding as we realised that he was getting reflux. Ben continued to have severe colic like symptoms and when he was 10 weeks old I broke down and we went to the GP. The GP gave Ben Lactulose, Ben needed Gaviscon for his reflux but this was giving him constipation so the addition of Lactulose helped to balance things out.

Until now we had been seen by a trainee Health Visitor who I had found not very encouraging. When she moved on we were changed over to see a qualified health visitor who had previously worked as a special care nurse. She came out regularly to weight Ben and chat to me, this was really helpful to have the appointments at home as Ben still would not eat and was easily distressed outside of his home. I realised that I should have asked for a different health visitor sooner.

At 12 weeks old Ben developed nystagmus in his eyes. We took him to the GP who referred him straight away to the eye clinic. We went that afternoon and waited for over two hours for our appointment. When we finally saw the doctor she was blunt and did not even introduce herself. Ben had fallen asleep in the hot waiting room and didn’t want to wake I suggested if I changed his nappy on her examination bed he would wake up, she looked at me as if I had said something disgusting and said it was pointless examining him because it was not possible to judge the vision of such a young baby. We went home worried and upset.

Around this time it was suggested that Ben see a physiotherapist, we should have been worried but we didn’t think anything of it. She came to see us and gave ideas on ways to hold Ben, settle him and make his reflux more comfortable and this helped to a degree.

Finally between 12 and 13 weeks Ben began to smile, what a joy. Still not at faces but usually to the mobile on his changing mat, Adrian had a game with him where the animals on the mobile would come and kiss him and Ben loved this and so did we.

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At about 3 ½ months I stopped trying to breast feed Ben and just fed him instead with expressed breast milk in bottles which he was able to take better. This cut down a little on the exhausting feeding routine. Adrian had bought me an electric pump with which I could express both breasts at once cutting my expressing time down by half which was a real relief.

Aged 4 months Ben went to see his paediatrician in clinic for his follow up appointment and the physiotherapist joined us there. The paediatrician said that he would like Ben to have an MRI scan and would arrange this. The penny didn’t drop until I was driving home and I suddenly though, my little boy has brain damage. When I got home Adrian tried to reassure me but this appointment had had a different feel to it and although no words were said I was worried.

We started Ben on solid food at about 16 weeks as was the advice at the time. He slowly did ok and over the following months would enjoy eating baby cereal, apple puree, rusk and milk, many of the usual baby foods.


The first diagnosis

Just over two weeks after the clinic appointment I had gotten Ben and myself ready to go into town and the post arrived. I decided to open it quickly before leaving and found the letter from the clinic appointment we had attended. In the letter the paediatrician told the GP that he was concerned that Ben had cerebral palsy, another letter was also included for Adrian and I inviting us to come to the hospital to discuss these concerns outside of the normal clinic times. Even looking at that letter 6 years later I find it amazing how one minute everything was fine and the next our absolute whole world was changed by two words on a piece of paper.

[Dad says; At the point of the diagnosis we had already seen some very significant indications of brain damage, I had come to terms (as far as one could) with having a child with problems such as cerebral palsy, I reasoned with myself that Ben would be strong enough to live a relatively good life even if this was with disabilities and we would help him to enjoy any activities he wanted to pursue.]

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Frustratingly the paediatrician was on holiday and we were unable to meet with him for two weeks. When we met he explained that Ben had shown signs of distress during labour but that this was not dealt with. When he was born he was not breathing but was attempting to do so, gas from his umbilical cord showed that he had been asphyxiated but that this had not been passed onto the people looking after Ben hence he had been allowed to become dangerously ill.

We were told that since his birth the hospital had been running an investigation into the mistakes made and that the midwife responsible had gone off sick and the doctor had moved onto another hospital. We were also told that the hospital’s consumer relations department would be in touch with us and that we should seek legal advice about compensation. We were also told that Ben's placenta had been in poor condition.

In that room it was like an out of body experience, like watching something on TV not anything that could happen in my life, it was unbelievable. I was plagued for months afterwards going over and over in my head the events of Ben’s labour and wondering at which point I should have realised something was wrong, blaming myself for not making a fuss or asking more questions, I worried endlessly about the future and thought my heart was broken. [Dad says; For me, and I'm not sure why, this news was more devastating than any news we had about Ben's problems. Problems caused by nature seemed tolerable but caused by human error was very upsetting]

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A week later the hospital’s legal services team invited us to a meeting. They apologised for the treatment that Ben and I had received and explained to us what would happened if we decided to make a legal complaint. I found them patronising and was so tired that I hardly needed another appointment to attend. When we left they shook our hands and something about these people touching me disgusted me.

We spoke to a lawyer friend who suggested we contact an excellent firm of lawyers Leigh Day & Co of London. They were very helpful, listened to our story and took our case on claiming legal aid for Ben. I asked the hospital for a copy of my medical notes.

Around this time with news of Ben's diagnosis to my amazement Adrian and I both found that people we had considered to be our friends and who we had seen regularly socially dropped off. They stopped returning phone calls and emails it took a while for the penny to drop for me, I continued to send Christmas cards to them for four years when finally I thought enough was enough, they are gone for good.

Happily our true friends and family have been there for us which we are really grateful for, but the nature of this journey seems to be that we mostly have to walk this road together and alone.

 

Life with special needs

At 20 weeks I stopped producing breast milk, pumping milk was taking over my life and my body had now changed by producing too much. It was hard to go out because I needed to express all the time and although everyone says this is easy to do when out and about I didn’t find it so. It took me 10 minutes to express my milk six times a day so to reduce this with the minimum discomfort I had to time myself and express for one minuet less every couple of days so that my production went down gradually. After a couple of weeks of stopping I started to feel much better and was less tired.

I tried taking Ben to the local mother and baby group one day when he was quite settled as I was concerned I had so far had little opportunity to socialise him. The people at the group were mostly friendly but unlike the other children his age Ben just sat on my lap and could not do any of the things available at the group, after a while he fell asleep. Still quite fresh from the pain of his diagnosis I found this distressing and did not return.

Aged 6 months Ben was referred to the speech therapy team to be seen in their feeding clinic to help with his feeding. Ben also had all his routine immunisations.

Also at 6 months Ben had an MRI scan. He had to be sedated for this and it took a while to settle him down. A couple of weeks later it was time for another communication. Ben’s paediatrician (who we still see today) had allowed me to email him when I had any questions and concerns. One evening I checked my email and a message from him told me that Ben’s MRI results had returned, it had shown extensive areas of brain damage. I had known by now that this must be true but to read it in black and white was still shocking and so hard to take in. The MRI report also stated that the damage was consistent with an HIE episode, confirming that Ben's birth suffocation was the cause.

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Still exhausted from looking after Ben we inquired whether there may be any kind of respite to help us look after him. We inquired with social services as did our health visitor but we were told there was not. We tried paying people we knew to baby sit but Ben would not feed for them and some found him too difficult to look after. I have little family , my Mum lived too far away to help very often but started to come round for 4 hours once a fortnight to help me clean the house.

Ben continued to have feeding troubles, when I fed him he would go stiff after a while, his body would go straight and he would push backwards really hard. He was started on the medication Zoton but I later realised that he never really took much of this as the granules were too big for many to pass through his bottle teat. Ben’s head had started to grow big in proportion to his body and this was being monitored.

We decided to take matters into our own hands with Ben's poor feeding. We found that an organic make of baby food produced jars of cocoa and vanilla pudding that had good nutritional value and good calorie content. We started to buy these jars in their dozens and put some in Ben's bottles of milk.

 

This thickened his food helping his reflux and also gave him the experience of different flavours. We passed on the nutritional values of the food to our dietician who approved. In time we would also make our own prune puree to put in Ben's milk and liquidise bananas to add to it too. We bought the biggest size teats possible for Ben's bottles and we fed him fairly successfully like this for a couple of years although reflux and sickie burps continued.

We were also reviewed in eye clinic and told that Ben needed glasses so a tiny pair of glasses were ordered for him. I encouraged Ben to keep these on all day, I so desperately wanted to stimulate his vision. They often slipped and seemed to block the top of his nose but I just kept putting them back on.

We advertised in the local shop and employed a cleaner to help me clean the house once a week, I felt guilty not being able to cope with everything, I had intended to be the perfect wife and mother. But we found a friendly lady who did a great job and was some welcome company for me also.

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I went shopping in town every few weeks when Ben needed things and often in the car park I would see a car that looked like the car of Ben’s midwife who I felt was responsible for his condition. I wondered what if anything I would say to her if I saw her.

During the summer Ben had a particularly bad spell of poor food intake. I contacted the hospital but Ben’s paediatrician was on holiday. I contacted the dieticians department who had also started seeing Ben via the speech therapy feeding clinic. Ben was drinking no more than 300mls a day and the weather was so hot. The dietician would not speak to me because Ben’s notes were away with the hospital’s lawyers. I went to the GP but they could give me no advice, I wanted to know how little fluid it was safe for Ben to take each day before I should be concerned about him becoming dehydrated. I contacted the dietician’s department a few more times but he would not speak to me due to the lack of notes despite me explaining to the receptionist my simple enquiry.

Finally I phoned the hospital’s legal team who we had met those months before and explained the problem with needing professional advice and Ben’s notes not being in the hospital. The woman we had met at our meeting told me that the notes would probably be away for some time because the legal team where they had been sent were very busy and that they probably only managed to photocopy a few pages of Ben’s notes a day. I had been trying for days to get Ben help and I lost it and uncharacteristically for me shouted down the phone at her “do you not realised you have ruined our whole lives, do you mean to tell me that my little boy can’t get help because someone doesn’t have the time to use a photocopier, why don’t you copy them?” I was shaking, crying and furious, I couldn’t believe the stupidity of it. The notes still did not turn up, another week passed and Ben’s feeding picked up again all on its own.

Around the age of 10 months the portage service came into our lives. This was a lovely lady introduced to us by our physiotherapist. She lent us some fibre optic lights to teach me how to stimulate Ben’s vision and other simple toys to encourage Ben to use his hands. It was great to be getting some professional advice to top up and back up all the things we had been trying to do ourselves. A few weeks later we also began to work with the sensory support team, a teacher especially trained for children with visual impairment who worked for our county council.

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Ben was also reviewed in the eye clinic aged 10 months. He had been going regularly, first the orthoptist checked his vision in his glasses and sometimes he saw the consultant. On this occasion the orthoptist checked Ben and then called the consultant in to see him. The consultant came in without speaking to me, sat down and looked at the notes the orthoptist had made, then he turned to me and said “you have had his brain scan results now haven’t you?” I replied that we had, he said “well looking at this there’s no telling what he will ever see” and he got up and walked out of the room. I was stunned and drove home in the pouring rain realising for the first time that my little boy may never be able to see my face or his own beautiful face.

A couple of weeks later we had a happier day. Our portage worker had arranged for us to visit the sensory room at the children’s clinic that was part of the hospital. It was a small room filled with sensory equipment, it was a revelation. Ben relaxed on the warm water bed and responded to the fibre optic lights, glow wall and bubble tube. After all year having heard all the things that Ben could not do it was fantastic to see his response in the environment, I went home and phoned Adrian straight away to tell him all about it.

On the information board at the children’s centre I had read about a group called Musical Keys, a music group for children with special needs. We started going to the group once a week and were happy to find it welcoming, they accepted us for who we were and Ben was able to join in as much as he could. We met other families and made good friends there. I was pleased to be able to be taking Ben out to a social group and doing something together that we both enjoyed.

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In November I was carrying Ben upstairs to our lounge which was on the first floor of our house. I tripped on a stair and fell onto my knees, Ben fell forward still in my arms and luckily I got my hand behind his head to protect him as we both fell forwards with a bump on the landing in front of us. Although I had saved Ben from most of the impact his right arm had flown out and landed with a wack and the experience of the fall was very frightening for him.

He went into shock and couldn't stop vomiting and became pale and unresponsive. We phoned the GP who sent a paramedic who took us to hospital. This was our first trip in an ambulance. In A&E the doctor did not know what to make of Ben and had to ask us if his stiff muscle tone was normal, actually we thought Ben a bit floppy at the time! Ben's arm was bandaged and we were sent home after a couple of hours. The local Lions Club had bought teddy bears for children attending A&E, Ben received a red teddy bear, this was also our first experience of the fantastic work of The Lions Club.

 

HYDROCEPHALUS

Aged 11 months Ben’s head size had increased dramatically. He was referred to a Neurosurgeon who said that although Ben’s MRI scan had shown adequate drainage of his brain his large head size meant that he needed to have a shunt. So at 11 months old just after our first Christmas together Ben had brain surgery and a vp shunt was put in. He wore the cutest little surgical gown to go to theatre, whilst he was there I sat and wondered if the people who had injured him had any idea what we were going through.

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Ben was in hospital for a week. I found it really difficult to sleep on a ward of young babies and children. One night the doctors were in our bay into the early hours of the morning with all the lights on congratulating one another on a fine piece of surgery performed on another patient and discussing golf! To keep Ben comfortable I had to hold him a lot of the time, the only pain killers he was allowed were paracetamol and he was in a lot of discomfort, my arm felt like it was going to drop off as there was nowhere for me to lean just a basic plastic chair.

I had to leave Ben twice each day whilst I went to the parent’s room and washed and sterilised his bottles myself as no one would help me and there was no one to sit with him. One morning I returned to the sound of his screams, he had somehow got his legs through the bars of the cot and was curling up and screaming in pain as the shunt in his head went down to an abdominal wound where the excess fluid could drain. No staff from the desk right outside his room had even bothered to check where the screaming was coming from. Adrian made the 2 ½ hour drive each day to visit us, whilst he was there I would go and buy enough food to last me until the next day and have a quick wash. From them on I have always found hospital stays like being in prison.

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The shunt has kept Ben’s head size normal since then and it is checked once a year.

 

Eye Troubles

I contacted the eye clinic several times by phone to ask for a bigger pair of glasses for Ben as his increased head size meant that they no longer fitted. As we had had a pair of glasses less than 6 months we eventually got a letter back from consultant saying that we could not have a spare pair of glasses. We had to write back a letter to him to explain that we were not asking for a spare pair of glasses but a new pair due to Ben’s head size [Dad Says; Unfortunatly we had got the point where talking with the hospital was not working so this was a very formal (strongly worded) letter!]. Contact with the clinic was nothing but stress and distress so I asked for a second opinion at another hospital. This has been a hard lesson learned for me but now when I realise that we are not getting the support we need from a professional I have the courage to ask for a change for Ben’s sake although I still find it hard.

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A while before his first birthday Ben had started pushing to stand on his feet, we played a game on the sofa together in the evenings, I would hold Ben on my lap, he would push to his feet and stand up and Adrian would give him a kiss, he thought this was a great game. He had been given a puppet for Christmas which was like a glove with a different farm animal on each finger, we found he enjoyed mimicking the animal sounds which he did quite well and was attempting to mimic words.

 

Chapter 2

1st Birthday

For Ben’s first birthday we had a gathering at home, so many people turned up that we could barely move in the house and we appreciated this show of support from family, friends and neighbours.

Throughout Ben's first year and into his second we regularly saw Ben's physiotherapist and speech therapsit, usually every 1-3 weeks.

Just into Ben’s 2nd year we began hydrotherapy. Again I was pleased to find something that Ben and I both enjoyed and could do together. I was taught how to exercise Ben in the water and we met other families there which was great. Ben really enjoyed the warm water

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Through Musical Keys we had found out about a group called School for Parents run by Scope which was supposed to be based on conductive education. We went along to meet them and here I had another revelation with Ben. I had been reading about conductive education which had been in the news at the time but however much I read I had not found a description of what it actually was, during our first session all became clear. Ben really thrived at this group and stayed there until he was the maximum age for the group of 5.

 

Moving

We had decided to move back to the area where we had previously lived, where there would be more schools and special needs groups for Ben and where we would be nearer family for support. Our lawyers who had been in contact with us over the months advised that we look for a house that was big enough for Ben as he grew older.

It took about 4 months of looking to find a house suitable in the area we wanted so when Ben was 15 months we moved house hoping for a fresh start. We were referred to all new services at the hospital in our new area but chose to keep our main paediatrician at the hospital where Ben was born as by now he had our trust and we had found that he listened to us which we had also found was rare.

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Eyes Again

Ben went to the eye clinic at the new hospital, when he was checked it was found that the glasses he had been given were more than double the strength that he actually needed. The consultant advised us that although Ben seemed to have a tiny bit of vision the glasses would give him such little benefit that it was not worth him wearing them so we gave up glasses. In a way it was a relief because Ben had been distressed by the regular eye examinations that involved him having drops in his eyes.

Several medical experts funded by legal aid came to visit us as Ben’s legal case had reached a stage where their opinions were being sought for Ben’s future needs.

We had a blessing service for Ben in our new local church as we had not yet held a celebration of his arrival to the world. On the day of Ben's blessing service I noticed that he had a sore swollen big toe, this was the beginning of the long saga of Ben's ingrowing toenails.

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To our surprise we found I was pregnant with our second child and I started to feel quite ill. My sister in law who lived on the other side of the country had told us about direct payments and how some families were receiving 40 hours per week of help so we asked about this and our new health visitor who was very supportive referred us to social services.

Direct payments was quite new to Norfolk, we had to wait for someone to become available to assess us. When the so called assessment was done we were told we could have 6 hours a week help because that was the most that any other family was getting. After many weeks of my phone calls being un-returned I managed to establish that the person who had assessed us was not even a social worker but an occupational therapist.

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Many more weeks of me trying to complain and not being able to get through to anyone or not having my messages returned followed. Finally we were assigned an actual social worker but were stuck with our 6 hours a week help for some months we employed Ben's Grandmother who gave up her job in a care home to take on the role of Ben's direct payments carer.

We were also assessed at this time by the home based nursing team from our local children's centre. The team was sent up to give respite to families with children who had complex medical needs. With his diagnosis of cerebral palsy Ben was thought not needy enough to qualify for help from the team.

When Ben was 17 months old the pre-school liasion group started writing to us asking us to fill in a questionnaire about every 6 months. This was the beginning of pressure being applied to us about Ben's future education but I did not realise at the time how bad this would turn out to be.


THE DARKEST DAY

On evening Ben was physically sick and went floppy, we phoned the out of hours GP but it took them a long time to phone us back by which time we had settled Ben off to sleep, we put this down to a virus but think now that this was the first of Ben's seizures.

Medical experts continued to visit us as part of Ben's legal claim at 18 months old a specialist paediatric neurologist came to visit Ben at home. He examined Ben and also saw our home movie footage of him shortly after his birth. To our surprise he said that he felt there was another explination to Ben's condition other than birth asphyxia. We agreed that he could contact Ben's doctors to arrange some further test but when he had gone we agreed that we were not convinced.

Ben attended hospital soon afterwards and had urine and blood test taken, this was a long hot day sitting around the hospital waiting for Ben to produce a sample. We bought him a big red balloon to take home and didn't think too much of the testing going on as the previous MRI had confirmed asphyxia.

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Many evenings when Ben finally got off to sleep I would dash out in the garden that was in need of TLC if only for 15 minutes digging up prickly or dead plants, I wanted the garden to be a welcoming place for Ben to explore. We bought several sacks of bright daffodils and tulips, I was changing from a person who liked pastel colours to one who looked for everything to be bright and contrasting for Ben.

Around a couple of weeks later on a sunny evening we had just finished our dinner when the phone rang. It was Ben's paediatrician he said that he had received the test results and had a new diagnosis for Ben, he said he had doubled checked but the only thing that these results could mean was that Ben had a condition called Canavan Disease. He gave us some website addresses where we could look up this condition and said he would phone us again the next evening when we could ask him any questions having had a chance to digest the news.

Adrian went to the study to look up the information and I continued to sit on the sofa feeding Ben his bottle just thinking it was all odd and wondering what Adrian would find and then like a huge dread the realisation hit me and I knew.

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Adrian printed off the information and we read it together in tears. One website specified that children who have symptoms of canavan disease in the first 6 months of their lives die between the age of 3 and 4 years. Here was Ben in my arms 18 months old whilst I read this unbelievable information that was telling me that he was already half way through his life. We put Ben to bed and there we sat crying, numb, I can't even remember going to bed that night. [Dad says; This was truly devastating, I could cope with the prospect of severe cerebral palsy, but this is the biggest bomb shell I could possibly image. I could not sleep that night as my heart was racing and my body was panicking about something that was now out of our control.]

So there it was. I felt like we had been giving this amazing gift of a little boy, the little boy of my own that I had dreamed of since I was 13 years old and bit by bit he had been taken away from us. It seemed there was nothing else they could take from him now.

I think I cried all the time for two weeks. Each time I walked into the utility room I saw the sacks of daffodils and tulips stacked up to make the garden bright for Ben, but how could I plant them now and watch them come up year after year without him? Every night I cried until there were no more tears left and fell into an exhausted sleep, thinking the darkest of things, where would we bury him? What could we possibly right on his grave stone that could sum up how much we loved him? Would he suffer when he died?

If ever I had thought I had known grief, pain, hopelessness or love I now knew that I had not.

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Whats more this new diagnosis this word that no one had ever heard of left an uncertainty hanging over the baby I was carrying, would it to have this terrible disease and be born only to be robbed from us?

I suffer terribly with morning, noon and night sickness when I am pregnant and this period was a huge struggle.

Adrian, Ben and I went to hospital to meet with genetic counsellors and have blood samples taken for DNA testing so that our unborn baby and families could be tested for canavan disease.

My gene fault was identified but Adrian's was not (he still has it but it is not known which gene). This was enough information for a 99% accurate amniocentesis to be carried out on our unborn baby. I am scared of needles but the amnio was not as bad as I had feared, the only thing that actually hurt was the small injection of local anaesthetic before the procedure started.

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I was 16 weeks pregnant before the result came through, the baby would not develop canavan disease and it was a girl. We heaved a sigh of relief although still numb and grieving. I hoped that Ben would live long enough to meet his sister.


BEN AND BUMP
What I found in my grief was that life takes over. I was going to hospital myself for a scan of the new baby every 2-4 weeks, the babies growth was being monitored as Ben's placenta had been in poor condition. It was agreed that I could have a cesarean delivery at my request, I didn't like the idea of surgery but I felt I could not risk the baby by going through another labour.

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Ben continued to have many appointments, therapies and special groups almost every day so life was busy busy. My step father kindly came and planted the daffodils and tulips for us.

Ben's legal claim had fallen flat, it turned out that the person who had reported on his MRI scan back when he was 5-6 months old had not looked closely enough, if they had his true condition may have been picked up much earlier. There was no way a doctor could now stand up in court and claim that Ben's condition was only due to his birth treatment when he had canavan disease.

Our health visitor who was a great support and our key worker wrote to every one that was involved with Ben explaining his new diagnosis so that we would not have to. This was the only way in which the key worker scheme worked for us and we opted after the first key worker meeting not to have anymore.

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With Ben's new diagnosis the home based nursing team from our local children's centre now granted Ben 3 hours help each week, a nice lady came and sat with him whilst I did chores around the house.

We had been advised to buy a house big enough for Ben to grown into, it was going to be adapted as part of our legal claim but now it was down to us. We contacted our council about a disability grant but they said it would take 6 months to get an answer. In the mean time baby was on the way and I had another fall on the stairs trying to carry a stiff Ben up to bed. We felt we had no choice but to adapt the house ourselves.

The house had an annex attached so work started on knocking through to it to give Ben a downstairs bedroom and bathroom our social services OT checked our plans. Having builders in the house, being pregnant and looking after Ben with little help was very tiring.

One morning I was sitting giving Ben his bottle as normal when he became unresponsive, staring, I didn't know what to do and phoned Adrian, then his face began to droop and he looked like he was having a stroke. I should have phoned 999 but I stupidly didn't have the confidence to. I phoned our GP who arranged for us to go into hospital. On the way to hospital Ben was sick and then he fell heavily asleep.

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He slept the whole day in the hospital, with a big bump now I had to sit with Ben asleep on my lap for hours over a cardboard bowl to catch a urine sample. Finally in the late afternoon he woke up and took a bottle. We were allowed home without any explanation. I felt sure though that Ben had had a seizure.

 

 

NEW WHEELS

A few weeks before our move to Norwich we realised that Ben needed a special buggy or wheelchair. The three wheeler buggy we had carefully chosen when we were expecting him had not been suitable for long, as soon as he had grown and needed to sit up in it he flopped over to the side or his bum slid forward in it and he hurt himself on the straps. Our physiotherapist told us that we must get a special buggy to give him adequate support or his spine would become deformed with bad posture.

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This was our first frustrating experience of needing specialist equipment. With our professionals knowing that we were moving we were referred to wheelchair services but unknown to us it was the services in the area we were leaving. The day before we moved we attended a special equipment show and tried out some buggies, I thought these were great and was so pleased to find something that would mean we might start to be able to get out and about a bit with Ben, I was worried about his spine so couldn't wait to get one.

When we moved after some weeks we met our new OT and found out that despite weeks of waiting we had to be re-referred to the wheelchair services in our new area, they would not give us any allowance for the amount of time we had already been waiting for an assessment despite the re-referral being requested as urgent.

If we tried to go for a walk with Ben, which he was beginning to tolerate he just got uncomfortable in his ordinary 3 wheeler buggy and would groan and groan and we had to stop literally every few paces to adjust his position, despite padding the chair out with cushions and rolled up towels for support. So here we were wanting desperately to get out just a little bit after over a year of being housebound but we could not take Ben out because the buggy we had caused him pain and professional advice was that it would damage his spine.

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This was a very frustrating position to be in, we were having a taste of "the system" that was to become our life, not what we had had planned. Eventually Ben got an assessment at wheelchair services. They showed us a chair for Ben that they would have provided, this to me looked like a basic plastic chair, the kind you might find in a village hall on a basic tubular frame. It was not possible for it to have a hood or a rain cover or anything that would make it practical or suitable for a young child vulnerable to temperature, this buggy cost around £1000.

Then we saw two other special buggies, the ones that we had already seen at the equipment show but these buggies with hoods, rain covers and essential accessories that you might find on a child’s buggy and cost around £2000.

Wheelchair services would give us a £1000 voucher and we would have to find the rest of the money to pay for the chair. There was no comparison and we asked for one of the special buggies to be ordered. Very fortunately for us at that time a family member was in the position to be able to offer to purchase this for Ben for which we were very grateful.

Before having a child with special needs I assumed that if a person (especially a child) in this country needed something like a specialist wheelchair to prevent their spine deforming then it would be provided for them. The reality is that most families have to beg charities to purchase such equipment.

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GASTROSTOMY BUTTON

When Ben was 22 months old the time came for him to have a gastrostomy button fitted. When this had first been suggested to us in his first year we thought it sounded very drastic but when we met again with the paediatric neurologist he advised that it was best to have this procedure done before Ben's canavan disease advanced and he was not as strong to tolerate the anaesthetic.

It was advised that it was very likely that Ben would need some assistance with his feeding in the future and that the tube would assist us to administer medicine. So we agreed.

We were seen by surgeons at our local hospital this time who advised that for a child with severe reflux like Ben when fitting a gastrostomy button it was advisable to have another procedure called a fundoplication. This procedure would tighten the valve at the top of Ben's stomach preventing the reflux so we agreed to this also.

The day before the surgery was planned we attended the hospital's pre-op assessment clinic, when the anaesthetist came to see Ben she was not happy and actually said to me "do you realise that he has a serious condition?" Actually we did! She said that the two planned procedures made the anaesthetic too long and that she could not allow the fundoplication aspect to be performed. We had no choice but to agree.

Ben had his surgery the next day, I was almost 6 months pregnant by now. I advised the team that for his previous surgery Ben had been gassed to sleep as it was difficult to find a vein to put Ben to sleep, unfortunately they did not listen. Ben became quite distressed in the anaesthetic room while he was prodded to try to find a vein, I had to hold him down, I was angry with myself for not being more insistent. Finally the doctors gave in and gassed Ben to sleep putting in the required IV when he was asleep.

I left the theatres and was given a pager which would bleep when I should return to the waiting room if I did not want to stay there for the duration of the surgery.

I can't remember now how long the surgeon said the procedure would take (hours) but that time came and went and I returned to the little waiting room. Another hour passed, other parents came and went and still no word about Ben.

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By now my imagination was getting the better of me, I knew the anaesthetic was dangerous, what if the worst happened now before Ben had even met his new baby sister? Time dragged by and my heart rate rose, finally I knocked on the double doors with the theatre reception on the other side and asked for an update but I was just told to sit down and wait. My heart was racing, I began to feel out of breath, dizzy and faint finally someone came for me.

Ben was in the recovery room, he was very sleepy but was ok, the relief was huge.

The doctors wrote up a feeding regime for Ben, they wanted to ensure that the gastostomy worked before they allowed him to go home. The next morning the nurses came and tried to administer Ben's usual medicine Zoton that he took at that time for reflux in his bottle of milk but today it was via his new gastrostomy. But the medicine contained fairly large granules and the tube was quickly blocked. The nurses had to remove the tube that was taped to Ben's newly inserted gastrostomy this made him scream and cry in pain. I thought afterwards that someone should have anticipated that this would happen.

The days passed and the gastrostomy feeding was not going well, The nurses administered Ben's gastrostomy feed as per the doctors instructions but as the fundoplication had not been done the milk came up and straight out of Ben's mouth, he screamed and cried in fear and pain choking. I had to hold Ben still whilst the nurses continued to try and feed him this was terrible.

We were in hospital 5 or 6 days. On the final morning Ben woke early and began to cry in hunger. I alerted the nurse at 7am knowing that Ben had to have some medication before he was allowed to feed. I waited and waited and ask again. I held Ben in my arms to calm him, this was hard physical work because he was stiff and straining, still no one came so I asked again, always polite.

Finally at 10am I had had enough we had waited and waited and Ben was now distressed and straining so much that he was causing himself pain due to just having had surgery. I realised that Ben's travel bag was in the corner of the room which contained an emergency bottle of milk, I got out the bottle, put in some Infant Gaviscon which I always kept in the bag, warmed the bottle in the sink and gave him the bottle. He nearly bit my hand off he was so hungry. He drank the full 200mls of milk and then fell asleep in my arms exhausted.

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A short time afterwards the consultant came on his ward round and asked for an update on Ben. I was exhausted and could take no more and broke down in tears. He asked what Ben had fed that morning and I told him about giving Ben a bottle, the nurse said little covering up the fact that she had left him crying and straining for 3 hours without bringing us a feed with no explanation.

I insisted that we had only had this procedure done at this stage whilst Ben was young and still in stable health, he was able to bottle feed so I should be allowed to take him home and try the feeding at his own pace. After some persuading the consultant agreed that Ben could go home later in the day.

In hospital I felt trapped and unable to care for Ben properly I had no control as to whether his medication and feeds were on time or to provide him peace and quiet or a comfortable temperature to rest.

During that final day we waiting around the hours that for some reason it takes discharge papers to be written and our medication to be returned to us from the cupboard in which it is locked. After 5 days of little sleep in a noisy hospital and being physically exhausted from having to hold and comfort Ben in an effort to prevent his straining in pain causing him further injury when I was pregnant I felt like a zombie.

Someone from the dietician’s office came to see me to explain the workings of the feeding pump which we were given to try to give Ben overnight feeds with. I could not take in a word she was saying and just nodded, feeling very worried underneath and hoping there were good instructions in the booklet she also gave me. Finally we went home.

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In the following days we tried to pump feed Ben at night. The pump turned out to be a simple machine, nothing to be worried about. The paediatric neurologist who had initially convinced us to have the gastrostomy fitted had suggested that we slowly pump feed Ben overnight with the feed finishing about 4am so that Ben would wake in the morning at 7am feeling hungry but his overall intake in a 24 hour period would be increased.

This is a great theory! The trouble was when the pump finished at 4am it went off with a loud bleep waking Ben. Then he would be awake for many hours into the morning or he would need more melatonin to help him back to sleep which might work after an hour or more but would leave him tired for the rest of the day. This was not practical. Also the overnight feeding or any daytime feeding whether pump fed or boluses caused Ben reflux and he was sick 95% of the time.

Despite the bad experience of the hospital stay in the long run we were glad that Ben now had a gastrostomy. We gave up on tube feeding at that time. But we did begin to give Ben his medications via his gastrostomy which although not full proof did increase the success rate of administering medication. Ben was prescribed a more effective medication, Losec (omeprazole) for his reflux.

Another bonus was that if Ben had a seizure we learnt that we could give him a very slow pump feed. One of the problems with seizures that were happening in the mornings about once a month and would knock Ben out for at least 10 hours. Meaning he would wake late in the evening for a feed and want to feed all night, it would then take at least 2 weeks of disturbed nights to re-establish Ben's bedtime routine, leaving us all tired out.

Overall the surgery was a success but the on-going lesson we would learn with Ben was that he would do things in his own time, not to the plan of us or any doctor.

In hospital Ben had been withdrawn, wanting to sleep all the time, shutting down and was a sad little man. When we got home as soon as we walked through the front door he gave a shriek of joy. He woke up, perked up and started talking to us, smiling and looking around, we all laughed, he knew he was home I welled up with sympathy and love for him.


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OPEN ACCESS

Ben's paediatrician suggested that he was given open access to the children's assessment unit. This meant that if Ben became unwell I could phone the unit and they would give us advice and if need be arrange for us to come straight into the unit to be seen. This was much better than phoning the GP out of hours service or going into A&E as Ben got straight to the people he needed without delay.

Finally the building works on our house were finished and just two weeks before Christmas Adrian finished putting down new floors so we could move back into our lounge and start moving into Ben's new sensory room and bathroom.

 

Chapter 3

HARRIET ARRIVES

I was heavily pregnant now, five weeks before my due date I went for my next check up growth scan. The baby was growing well and the obstetrician said I could now come back on the day of my planned caesarean.

I was not happy about this, as Ben's placenta had failed in labour I wanted to ensure that this baby was ok right up to the end. The consultant agreed that I could have one more scan in two weeks time for my peace of mind but was sure that all would be ok.

Ben had his second birthday and we had a much smaller open day at our house with just a few relatives dropping in. I was just one month away from my due date now and on our home movie of Ben's birthday I look enormous!

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Three days later the two weeks had passed and I went for my next scan. During the scan the radiologist was concerned, I waited as usual to see a doctor for my results. The scanned had showed that the baby had lost weight and she did not move at all while I spent the rest of the morning on a monitor although the pulse through the cord seemed fine. Baby Harriet was delivered that afternoon by emergency caesarean.

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We were in hospital 6 days, Ben came to visit after two days but as it had not been long since his surgery he became anxious in the hospital and did not even want a cuddle from me.

On leaving the hospital we bumped into the doctor who had scanned Harriet 6 days earlier he said "Mrs Fletcher I can tell you if your babies placenta had not died then it was about to". I realised that if I had not insisted on having another scan then the baby’s poor condition would not have been picked up and she would have been dead by the time the consultant had next wanted to see me.

I was very glad that I had had the confidence to make a fuss and trust my instinct. Harriet was 4lbs 3oz she had rolls of skin under her neck and on her tummy as she had been losing weight, the placenta had not been feeding her.

I had severe pain after my surgery and wondered why anyone would ever opt to have a caesarean. I didn't know then but this pain is not normal, I had an infection but no one ever realised.

The day after we came home Ben had another seizure, we had to call an ambulance and Adrian went with him to hospital, we had only all been together a few hours.

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DOUBLE TROUBLE

Ben still bottle fed every two hours throughout the day, it took an hour to feed him. Harriet bottle fed pumped breast milk every two hours day and night. Harriet had her feeding tube in her nose for about 2 weeks before she was able to manage without it.

[Dad Says; As Sally had had a caesarean she couldn't pick up anything including Ben. For this reason I would attend to Ben during the night and Sally would do Harriet. Unfortunately Ben liked having me around too much and if he woke up after 3am he would not go back to sleep. This meant that I would need to get up early every morning, usually between 3:30am and 4:30am, If I was very lucky he would let me sleep until 5:30am. I'd then have to go to work at 7:30 once Sally had got up and be home at 5:30pm. The evening would then be non stop feeding until Ben was finally asleep at around 9pm when I would then go straight to bed.]

When Harriet was two months old it was suggested to us that we might like to consider staying at the children's hospice for a rest. Rest sounded like a good idea. We went to visit the hospice, it's an hours drive and I was so tired. It was a nice place but I realised straight away knowing Ben and the difficulties he had settling to sleep at all, let alone when away from home that he would not settle here.

During this same month Ben had two more seizures, we were all exhausted.

Since Harriet's birth we had had a mixture of direct payments, homecare and home based respite nursing. Social services came out for a meeting when Harriet was around 10-12 weeks old. This meeting that the others involved have no doubt long forgotten has changed me forever.

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At the meeting that took place in the lounge of our home were myself, the homecare manager, our current social worker and a social work manager that had been brought back from retirement because the department were so short staffed. They told me that my hours of help would be reduced to just 18 a week and that even this would not be maintained for long.

I explained that this would not even allow for Adrian to go to work, that both children still fed every two hours, taking an hour each to feed due to their medical difficulties, I explained that I could not even keep them fed without help let alone bath them and changed nappies, all the things that young children need.

Our own social worker sat there in tears, but the social work manager who led the meeting was firm she would not be giving us any more help no matter what I said or how I tried to explain that it would not be physically possible for me to care for the children no matter how hard I worked.

After the meeting I was left in shock, disbelief and numb. How could these people come into our house and decide that we did not deserve to survive? In the next few days and nights my mind was whirring I could not sleep with the worry going over and over how could I manage.

I should explain that we had no other possible avenues of help such as from family or friends other than the help my mother was already giving us, social services knew this well.

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The days went by in a blur, if I had to drive Ben to an appointment I did not feel safe doing so I was tired. When I looked down at the laminate floors in our home the patterns on them seem to crawl towards me because I was so tired. I phoned my social worker, desperate and pleaded with her again, telling her directly that I could not cope, I could not keep the children fed, she said "I am sorry Mrs Fletcher but there is no more help". I was pushed over a line, I felt like I was shaking all the time, I was sick with worry.

During this couple of weeks I became dangerously mentally ill thinking that there was nothing that could be done and that I could not cope. I believe I nearly died.

Then one night instead of going to bed at 8pm I sat up until 11pm writing a letter. I had a leaflet that I had kept about making a complaint to social services, I read it and wrote a letter of official complaint to social services. I detailed the services we had been receiving and the hours that had been removed, I explained Ben's medical condition and Harriet's, I explained that I could not cope, finally I went to bed.

In the spring those bright daffodils and tulips came up in the garden, the ones I had been afraid to plant after Ben’s diagnosis. Happily he has enjoyed seeing them for many years now.

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LIFE'S A FIGHT

The letter was received by the complaints department who acknowledged it and passed it onto a more senior manager in the children's department. Around two weeks later the manager came out to see us and talk to us, he saw the children and got to speak to me and Adrian.

By the end of the week he had granted us four days a week care from social services and had our home based nursing increased to one full day a week, we now had 5 days a week care enabling Adrian to go to work and me to have enough pairs of hands in the house to cope.

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We had no actual respite but would could care for our own children. The care was granted to us for 6 months, we breathed a sigh of relief. I dropped the complaint because I was exhausted from fighting and I had the result that I needed but in retrospect I should have pursued it. I believe I was pushed quite literally to the end of my tether.

Ever since this time I have feared meetings and had a strong dis-trust of social services which over the coming years has further been reinforced by their actions.

Ben continued to enjoy his groups at Scope and Musical Keys but with his new diagnosis I again felt a bit isolated. It was a real privilege to meet the children and families that we met at these groups and see them grow and progress, they were amazing, but now I knew Ben would not progress with them in the same way.

Ben continued to have seizures, he went for an EEG but this was normal. We had managed to video one of the seizures and sent it off to the paediatric neurologist nearly a year earlier but he had not managed to play it for some reason and we could not convince the doctors that Ben was actually having seizures.

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A few months later we had a different home based nurse for our one day a week. She was friendly at first and I taught her how to feed and play with Ben. But after 2 weeks of letting her feed him I found that the bibs she had used were soaked with milk, Ben had obviously not been drinking but spitting the milk out.

When Ben bottle fed, when he needed to take a breath he actually just sucked the bottle harder and harder making it hard to judge when to stop feeding.

The following week she was holding Ben at his feed time and I asked her to give him to me so that I could show her how to give him a bottle again. She said "no I will feed him" I explained politely that I thought I needed to show her again how to feed but she would not pass him to me, I reached my arms out to him and she actually twisted around moving Ben behind her back so that I could not reach him.

I felt distressed and started to cry I said "you are upsetting me now, give him to me” so she reluctantly passed him to me. As I sat on the sofa and fed him she laid into me telling me that she had x number of years nursing experience and that she was not here to be mentored by me.

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I was shocked that she would be acting this way in my home with my child. I wanted to cry, how dare she, it was still two hours until Adrian would be home I had no choice to let her remain in the house in case of emergency as Ben had been having regular seizures.

The next day I phoned the home based nursing manager and tried to explain what had happened, I think the nurse may have already spoken to her, as I started to tell her about it she cut me short and said that the nurse would not be coming to my home again.

Upset I let it drop but I felt that I should have made a complaint. I felt this was wrong and that a person such as this nurse should not have been allowed to speak to me a vulnerable person in my home that way. Still tired out physically and emotionally I had to choose to save my energy only for the battles that were essential to fight.

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A short time after this incident the speech therapist came to see Ben. She said that it was now no longer safe for anyone except Adrian and I to bottle feed Ben because it was so difficult to judge safely when he wanted to swallow and when he needed to breathe making it too easy for him to aspirate any fluid he was fed. Ben's ability to eat the solid baby food that he had still been on was decreasing steadily.

 

LIFE GOES ON

We wanted to try the supplement calcium acetate because it was recommended by the doctors at the canavan foundation and many other families that we were in contact with were using it. Our paediatrician was willing to let us try. We tried the medicine for about 10 days but even the low starting dose gave Ben terrible stomach cramps that were of an unacceptable level of pain for him to be in so we had to stop using it.


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Until now we had been collecting Ben's prescription milk from our GP surgery dispensary. Our helpful health visitor often brought our supplies to us when she was coming to call. At a routine dieticians appointments we were told that it was actually possible to have this milk delivered. This is an example of sometimes helpful services being available that no one seems to tell you about. We had been travelling with crates of milk in hundreds of glass bottles in the car with our children, not feeling safe for over a year, we asked for it to be delivered direct from now on.

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Chapter 4

RIDING

I have found that if you have an idea of something that you might like your child to access then it's always worth asking. I knew of the disabled riding association and that they had riding sessions nearby for children from the special needs schools in our area. I love riding and it had especially meant a lot to me as a child to be able to learn, as Ben seemed to enjoy contact with animals I wanted to give him a taste of this.

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I contacted the association and explained that Ben did not attend special school so would not be put on their waiting list by the conventional route but wondered if he might be able to attend for a one off session. Safety being the priority they arranged for their physiotherapist to contact Ben's to ensure my suggestion would be safe and suitable for Ben. Happily they offered Ben a couple of sessions riding and we proudly watched as Ben had the opportunity to ride on a horse

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KEYWORKER?

We were still members of the key worker scheme, our health visitor was our key worker, she was excellent and said that she wasn't actually doing anything that she wouldn't have considered doing as just our health visitor anyway. We had only had one meeting but I had felt that we were going through the motions and that it was not comfortable or very respectful for me to raise any problems that I might be having with an individual department in such a public setting.

Without my consent a letter had been sent to all of Ben's medical professionals inviting them to another meeting. I contacted the key worker scheme and told them that I did not feel up to a meeting at this time and I was told that it would be held without me. I felt unhappy about this, now having a fear of meetings I didn't want all these people discussing my family without me being there to defend myself and I did not feel up to defending myself. I wrote a letter official requesting that the meeting was cancelled and it was.

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SEIZURE CRISIS

Ben was now three years and five months old. His seizures were at least one a month and still the doctors would not acknowledge that it was actually seizures he was having because they did not fit into the usual descriptions of an epileptic fit. I had been reading about other children with canavan diseases experience of seizures on the canavan web board. Some parents were writing that their children were having prolonged seizures that would not stop without medication.

One evening soon afterwards Ben had a seizure, but instead of stopping after 2-3 hours it went on and on, at 11pm I called an ambulance. Two ambulances turned up, the crew were very nice but of course had never heard of canavan disease and were presented with an unconscious boy with poor breathing and they had not seen a fit like it.

They took us to hospital where we went straight into the children’s assessment unit. A doctor came and saw Ben and gave him a small dose of lorazepam, an anti-seizure drug and Ben fell asleep. He was transferred to the children's ward in the early hours of the morning but after just on hours sleep there he woke and his seizure pattern started again, his seizures came in three stages and he was going through the pattern but instead of waking up at the end of it the pattern just started over and over again.

This went on all day. We were in a high dependency room. The nurse looking after us was also looking after another child in the room and this child's needs were such that she was not allowed to leave the room even to have a break. She was run off her feet and working very hard.

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I drew her attention to Ben's condition and I spoke to the medical doctors who came to see him about it. The medical doctors wanted to check Ben for infection and took a sample of spinal fluid from his shunt. No one seemed to take into serious consideration his canavan disease causing the seizure.

During the stiffness part of the pattern Ben was rubbing his gastrostomy button and making it bleed. This continued into the night, he was becoming exhausted when the seizure pattern started his heart rate was above 215 for at least an hour each time, a defibrillator was plugged in beside his bed. I had not slept now in over 40 hours.

I asked to see the doctor. When he came I broke down in tears, I didn't understand why the doctors couldn't just go to the internet like me and read about the symptoms of canavan disease. I insisted that I had read about other children requiring medicine to stop their seizures.

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The doctors did not want to give Ben any more medicine for his seizures because he was not diagnosed with having seizures and his paediatric neurologist worked at another hospital and it had not been possible to contact him. I said "look at his heart rate, this has been going on for 28 hours, are you going to let him die?".

The doctor decided to give Ben some more anti-seizure medicine and he fell asleep. The seizure did not come back but for the next few days Ben was so physically weak from the experience that he could not even cough. The hospital would not discharge us until the test results came back even when Ben perked up and became well again.

We spent a week in hospital recovering from an incident that should have never happened if Ben's seizures had been acknowledged by his doctors and given us medication for them.

Soon after this Ben was given the medicine phenobarbital that controlled his seizures. I believe that if I had not read about other parents experiences on the canavan web board then I would not have had the knowledge or confidence to stand up to the doctor and insist that Ben have some medicine for his seizure. I think it is very possible that if Ben's high heart rate had been allowed to continue that he may have died. Ben's test results all came back negative, he did not have an infection, he has canavan disease.

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MELATONIN AND ALLERGIES

The next month Ben was prescribed for the first time the medicine Melatonin to help him sleep. Finally we were able to get Ben to bed without hours of screaming causing distress to him and us. The medicine worked well, it's not a sleeping pill but a natural sleep hormone that everyone has in their body but apparently some people with cerebral palsy type conditions do not make it. The medicine may take 45 minutes to work for Ben but 98% of the time now, when he is well he will fall asleep calmly. I recommend this highly to parents of children with sleeping difficulties.

Aged 3 years and 6 months Ben had allergy tests. His paediatrician had been on a course about allergies and found that these often caused reflux. The tests were very helpful because they identified that Ben was allergic to wheat and egg and highly allergic to peanut.

Ben had often had an allergic rash appear on his face but the cause had not been identified. We now know that this had been when we had eaten or touched egg and then touched or kissed Ben. We got an epipen for the peanut allergy even though Ben did not eat it, just in case. I think it's important to remember that as well as having canavan disease Ben is also a little boy who may have other allergies and conditions as well.

A couple of months later Ben's stiffness had increased a lot. He had an appointment to be assessed for botox but it was thought that this would not be possible or helpful.

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STATEMENT

We continued to be reviewed by social services to assess our needs every 6 months. The senior manager had come to see us following our official complaint for a year and then we were handed back to a new social worker.

Since Ben was two years the topic of him going to school had often been raised but we had dismissed this as we did not want him to go to school sooner than he would have normally just because he had special needs. We also felt from what we knew of the education system for children with special needs that in Ben's case the usual way of caring for children would not be safe because his seizures were serious and very difficult for anyone to recognise, in fact even in hospital when his seizures had been witnessed by experienced staff they had not been recognised.

We had been advised by Ben's sensory support worker, a teacher for visually impaired children that we should be thinking about a statement of special educational needs for Ben because this was a long slow process and then we would have the statement ready should we need it. We thought this sounded sensible and agree for the statementing process to begin.

This process involves the education department writing to all the people involved in Ben's care such as his doctors and therapist and the special needs groups that he attended and asking them to submit a report about Ben's achievements and needs. All the reports are then combined into a document which would be his statement of special educational needs.

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When social services found out that Ben was being assessed for a statement asked to come and see us for an update prior to our official 6 month review meeting which was coming up. Our social worker came to our house and met me on her own.

She told me that when Ben had his statement that our care hours would be reduced and he could go to school. I said that Ben was not even school age and would not be for more than year, I said that as Ben was already a year beyond his anticipated life expectancy that we would decide what was best for his education and social needs when he outgrew the special groups that he attended and currently thrived at.

The social worker said that if we chose not to send Ben to school then that was up to us but had to pass on that her manager had said that as soon as Ben had a statement, whether he was school age or not then his care hours would be cut by whatever amount of time social services estimated that he should be attending school.

I was horrified I explained that if our care hours were cut I could not look after Ben safely and also if they were cut it would actually prevent me getting him to school because I wouldn't have the assistance I needed to get him ready. I had long been told by many professional people that I should not send Ben on school transport because of his need for support by someone who knew him well to keep his airway clear and keep him safe.

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It did not matter what I said the social worker was adamant that our hours would be cut and she knew well the meaning that I had taken from this conversation. Once again I was racked with worry, I would go through a few months of being able to look after my children with assistance and then another reason would be thought up to cut our hours.

I knew that it would actually be much more expensive for our county council to send Ben to school as opposed to providing him with care in his home, the social services department did not care about this, they only cared that it would relieve a bit of the strain on their direct payments budget.

I again became ill, my hands shook, I couldn't sleep, when I am under stress I suffer from back ache bad enough to wake me at night and all my aches and pains come to the surface such as an old knee and wrist injury. I knew I had to find a way to fight again.

I began gathering evidence on what it costs to send a child to a special needs school. I spoke to my GP, health visitor, physiotherapist and paediatrician about my concerns. Our worries was that Ben was thriving really well in the special needs groups that he was in and we did not want this routine disturbed.

But my biggest worry was that in a special needs school Ben would be looked after by lots of people who did not know him well enough and never could in the amount of time they would spend with him. They would not be able to communicate with him, spot the signs of a seizure and judge his very precarious feeding needs.

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Our professionals came to our aid and wrote letters to social services, when I retold the story to one of Ben's doctors who said I should consider going to my MP. The only thing I could think to do was to ring the education department and tell them that I wanted the statementing process for Ben stopped and they did this, I hoped it would be enough. I also wrote a letter of complaint to the social services senior manager who had helped us before.

When our official 6 months review meeting came around a few weeks later the home based nursing manager and Adrian were also present. The issue of Ben being forced into school and our hours cut was brought up, the social worker deigned that she had ever said that if Ben did not go to school then social services would guess how much time he might be spending in school and cut his hours anyway, but she had.

Our care was granted again for another 6 months but once again social services had caused me considerable worry and distress and then lied about it. From then on I would never have a meeting without Adrian present so that if I was lied to again I had a witness.

I literally feared the review meeting time coming round, at the meetings we felt like we were being talked to like bad parents, often patronisingly, we had to explain every detail of our personal lives. It was like they were trying to give us a kick up the bum but we didn't need it we were doing everything we could to manage because we didn't actually want helpers in our home but we knew we needed them.

How it can be allowed that professional people come into the home of people that have already proven they are working as hard as they can to care for a child that has a lifelong condition that is only going to deteriorate and grill them every 6 months in the way that we were spoken to I do not know. I just know it's not right.

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HAPPY HOLIDAY

We had our first family holiday together to Derbyshire. It was really hard work but amazingly the children slept all week except one night. We got out and about a little bit in the day but chose to eat all our meals in the lovely house we stayed in which had been converted from an old stable block.

The change of scene was really good for all of us even though we parents were shattered. Just being away from the demands of Ben's appointments, his post every day and the telephone that constantly range for him was a real relief and I started to think about how I might be able to improve things at home in the same way.

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Steadily over the last 12 - 18 months Ben's tube feeding had increased as his tolerance improved, his ability to eat any kind of solid food had been lost but we still gave him the option of a bottle of milk, over time he would not take milk in the daytime but still at bedtime. Eventually he could not take the milk at bedtime but still liked the teat in his mouth as he drifted off to slip, finally this autumn when I put the teat to his lips that he had previously found a comfort it just made him gag and after a few nights I realised this was not going to change.

We had let Ben make the transition from oral to tube feeding himself just reducing his oral feeding as he lost the ability, I don't think we had to make a terrible choice to do it, it just happened of its own accord.

As winter approached we caught a sickness bug, Ben could not keep anything down so he had a couple of days in hospital having his phenobarbital intravenously to keep the seizures away.

Ben started having a flu jab each winter.

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Chapter 5

FUNDOPLICATION

Ben still suffered a lot with reflux. I had read that other children with canavan disease had had the fundoplication operation that Ben had been refused when he had his gastrostomy button fitted. Our consultant referred us again. Our local hospital still felt that they could not give Ben this operation but instead referred us on to a bigger hospital that had a designated paediatric intensive care unit and Ben was put on the waiting list.

The hospital was 2 1/2 hours drive away, the same one that Ben had gone to have his shunt fitted as a baby. Adrian took us and we sadly waved him off as this time he would have to look after Harriet and not be able to visit as much.

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We always seem to have surgery in the winter time and the wards in the hospital are always busy with poorly children. Ben's surgery went well but as always after surgery he was slow to tolerate his feeding. After 6 days Ben and I were exhausted. I felt that no one was really helping me care for Ben I was having to chase up his feeds and medicines all the time to try and ensure that they were given on time and then they were left for me to administer.

The ward was noisy and Ben was desperate for rest and so was I. A patient was brought in to share our room in a great deal of pain and understandably made lots of noise. I had had enough, I had to get us home but the hospital did not want to discharge us, I knew that it may take weeks for Ben's feeding to get to the stage they wanted to see it at and it was not as if they were giving him any IV fluids or medicines. Ben was crying with tiredness all the time.

I self discharged him that day, I was told his was no longer of risk of infection from his surgery. Adrian came and collected us in the afternoon, Ben slept all the way home, all that night and almost all of the next day, then all the next night. I knew I had done the right thing in bringing him home and getting him the rest he needed.

Ben suffered with gas bloat after his fundoplication. This gave him severe pain for four months. When we tried to remove air from his tummy it often bled for a year afterwards. We went back to the clinic and saw our paediatricians who could not help with the pain, our local surgeon dismissed the bleeding. This was a horrible few months of Ben being in pain but overall in time his reflux was a lot better and he had stopped having sickie burps. Over the summer Ben gained weight well.

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MEETINGS

Since we had been granted four days or 36 hours direct payments this had been reviewed every 6 months. I now would not have the meeting if Adrian could not be present as I felt I needed his moral support and his presence to witness what we were being told. At the meetings the manager for home based nursing attended, our social worker, our social worker's manager.

It became apparent to me that at these meetings we were just going through the motions. When our needs increased we had evidence from Ben's medical professionals to back up what we were saying but the hours were often cut regardless. I felt that it was actually decided by social services what they were going to do before the meeting and then coming and seeing us was just a formality.

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This summer despite Ben having increasing needs our hours were cut by 4 a week, I could not be sure at the meeting table but I was already pregnant with our third child.

We wanted another baby and chose to have the baby tested for canavan disease and happily the test came back negative, the new baby would not even carry the disease. We chose not to find out the sex of the baby.

As our care hours had been cut by social services some of Ben's professionals wrote to support our case and after a couple of months our hours were put back up to 36 but once again whilst they were cut I had been exhausted and whilst I had morning sickness.


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MAKE-A-WISH

We made contact with the make a wish foundation. I had some things in my mind that I hoped I would be able to provide Ben with the opportunity to do in his life time, things that had meant a lot to me as a child and things that I wished for my own children, one of them was a trip to Disneyland!

We had had a successful holiday the previous year but a trip out of the country was rather daunting. I had tried to arrange this myself but making contact with Eurodisney and various modes of transport but found I was getting conflicting responses if any at all.

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Make a wish knew who to contact and came out to our house to meet us. They had the experience of making travel arrangements for children with special needs. We chose to fly to Eurodisney, at that time it was possible to fly from Norwich airport which was a short drive away for us. This was the quickest mode of transport for Ben.

Some parents that we knew who had taken their child in a wheelchair to Eurodisney earlier in the year suggested that we ask to book some assistance to get us through Paris Airport, we asked Make a Wish if they would help with this and we were glad that we did.

The journey and taking care of the children on our own was hard work, especially as I was 17 weeks pregnant and just coming to the end of the worst phase of my morning sickness. However many moments of the trip were magic and I know I will always remember it so am very grateful for the experience.

The captain of the airplane welcomed Ben onto the plane over the loud speaker, all the other staff were great and really went out on a limb to help (the company was 'Flybe'). One of Ben's favourite toys had been his Winnie the Pooh bear which he'd had since he was a couple of months old. It was arranged so that Ben could go and see the Winnie the Pooh show and then meet the cast. I am really glad we made this trip although I felt so drained at the time. I think part of living is making memories.

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STARTING TO MAKE CHANGES

Ben was registered blind, although we know that he can see some dark and light and a little movement to an eye specialist he is blind. We did this because this meant that Ben could become a member of the Living Paintings library of special books for blind people and we started using this service.

In addition to the paediatrician that we had kept as our main doctor at the hospital where Ben was born we also had been seeing a paediatrician at our local hospital since moving, this had been helpful when we had first moved to connect us to local services but now it was duplicating our appointments.

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We asked if we could stop seeing the local doctor and keep our main one now that we were well settled into the area. We also asked if our local paediatric neurologist would see us rather than sending us to the one we had previously been seeing the 2 1/2 hours drive away. This helped keep Ben's appointments to the minimum and closer to home.

 

SCHOOL BEN'S WAY

Ben was now 4 1/2 and we had started thinking about school. We knew that once he was 5 Ben would no longer be able to attend Scope or have the Norfolk Portage Service visit him at home although he would still be able to attend Musical Keys and have sensory support at home.

We wanted to find something else that Ben could attend once a week for social contact, we felt we were doing a good job meeting Ben's development needs at home and this was confirmed by his professionals. We knew that two outings a week was just right for Ben, more than this and he became overtired and more at risking of a seizure.

I had been speaking to several of the special needs schools, additional groups and the home education department for many months as well as other parents. We had been discouraged to approach main stream schools as Ben had complex needs but I had been wondering about our small village school and Adrian encouraged me to make contact.

I arranged to go to the school and meet the head teacher with Ben. I explained that I was looking for somewhere as local as possible for Ben to interact with other children just once a week for two hours, I explained that myself or Ben's Grandma would always accompany him and stay with him because we felt it absolutely necessary that someone who knows Ben well is with him at all times to keep him safe.

Happily the head teacher was very open minded about the possibility of Ben coming into his local school and suggested she discuss this with the school governors. On the second week of term in the new school year Ben started attending our local primary school one morning a week for two hours as a special visitor.

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I had written a little bit about Ben which the head teacher read out to the other 45 children in the school. The children were curious about the ways in which Ben is different to them but children are amazing, they just accepted him for who his is, many of them being extremely caring and all of them being considerate towards him.

Ben's statement was applied for again and later in the school year he registered as a pupil at the school. Either Ben's Grandma or I always accompany Ben with the other one of us staying at home to care for his younger siblings, this way we do not need to worry about getting funding for a special helper and most importantly Ben is with someone who knows how to care for him and help him join in to the best of his ability with the other children.

The school have been fantastic ensuring that the parts of the curriculum most suitable for Ben are taught on the morning that he attends. I cannot speak highly enough of the open minded and encouraging approach of the staff to all the children at the school or the children themselves.

 

INNOCULATION

I had read on the canavan web board that some children in America were inoculated against chicken pox. I asked if it would be possible for Ben to be inoculated especially now that he was mixing with school aged children. Our GP surgery ordered an inoculation for Ben and he had it at the surgery.

Our GP surgery have been another source of support for us. They always fit Ben into surgery if I am at all concerned about him and the dispensary is very helpful with all of Ben's medications. I think different families find support amongst different professionals I think it's important to take support wherever you can find it and never hesitate to call of those services to see if they can help when problems arise.

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GETTING READY FOR BABY No3

We went to stay at the hospice three months before the new baby was due. We wanted to see if it might be possible for Ben to stay there with my Mum whilst I would be in hospital having another caesarean with the new baby. Unfortunately the night did not go well for Ben, even though he was older this service regrettably still did not work for him.

Happily by now Ben's life expectancy he changed. He was almost five years old so beyond the 3-4 years we had originally been advised. Currently it is advised that the majority of children with canavan disease die before the age of 10 years old but some now live into their teens and occasionally their 20's. I am told that this change is due to gastrostomy feeding becoming more common, certainly if Ben were not tube fed we would not be able to feed him at all and he would perish.

As a precaution Ben started taking a low dose antibiotic over the winter time. Ben was relatively stable, every week or two he would have some tired or unwell days, usually for no apparent reason and then he would bounce back.

I knew that the new baby would be our last and I wanted things to go better with the delivery than my previous experiences. I asked if I could meet with the consultant anaesthetist who would be responsible for my care, I had to be insistent that I really wanted to do this and I was referred and this was very helpful.

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We asked for extra help from social services for when I came out of hospital post-caesarean. The direct payments department did not give us any extra help but simply maintained the care that we had been having. The homecare department did not have any staff available to provide extra care so they instead gave us a direct payment which we could offer to our current care staff to work the longer days. The home based nursing team increased our care by an hour a day. This was all for when Adrian returned to work after his paternity leave and I was still unable to lift Ben having had a caesarean.

Ben had been assessed for hoists but the slings supplied did not fit him, in fact our baby would be several months old before the correct equipment was supplied to enable us to hoist Ben.

 

SAM ARRIVES

This time I made it right up to the day that the caesarean was planned. We went drove into hospital early in the morning, settled into a room. Were called to theatre, I was very nervous. The team started the delivery and it didn't seem very long before they all said in unison "Aaahh" and a few seconds later we heard a baby cry. It was a boy, Sam, he was lifted straight onto the scales, cleaned up a bit and brought to us he weighted 6lbs 3oz. It was amazing, neither of the other children had been strong enough to cry at birth and I cried with surprise. It was just like having a baby, just like you see on the telly. I felt like after all this time I had actually had a baby.

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The next day I was up and walking around my room, Adrian was amazed compared to last time I had just a fraction of the pain and now I think caesareans are great.

Again I wanted to breast feed I fed the baby and expressed milk to help bring my supply in but I did not produce much milk. I asked for a bottle to top the baby up but was told I could not have one as it would reduce the chance of successful breast feeding.

I was feeling very tired and struggling to wake at night to feed Sam, he was also tired and was not waking to be fed. I asked the midwife for help but she said I should let him sleep. After 3 days of this Sam was found to have low blood sugar and we were not allowed to take him home [Dad Says: We'll not go into detail of the strong discussions we had with the doctor and nurse following the low blood suger discovery!]. I was angry with myself for not insisting that Sam be allowed formula milk to top up his breast feeding.

 

HO HO HO!

Sam was born two weeks before Christmas; Ben was soon to turn six, once I had wished that Ben and Harriet would get to spend one Christmas together now we had a Christmas with 3 children. Life was hard with a baby again but Sam only fed 2-3 times at night. This time I pumped breast milk for just 16 weeks realising my limitations.[Dad Says: life is now feeling a bit more normal! but still 3 kids are very hard work.]

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Chapter 6

MORE SEIZURES

Ben's seizures continued to increase. I put a post on the canavan web board and found that many other children took a combination of medication for their seizures. I sent a message to Ben's neurologist and he kindly phoned us over the Christmas holidays and prescribed the drug Tegretol. Since the introduction of Tegretol with the Phenobarbital Ben has had much better seizure control, as Ben grows the seizures start to break through every once in a while and then we increase the dose as needed.

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A month later Ben had a large seizure, we now had the medication Buccal Midazolam to use at home but the first time it was used we had been asked to call for medical assistance. We called an ambulance and they watched as I administered the medicine.

It was one of those typical mad days that creep up and bit us on the bum every once in a while. Just as the ambulance pulled up so a huge Tesco's shop was delivered. Harriet was in bed sleeping and the baby needed feeding. My Mum was with me so I had to send her off in the ambulance with Ben while I sorted out the shopping and cancelled the planned appointments for that day. Now that Ben had used Buccal Midazolam safely at home we have been allowed to use it and administer it ourselves.

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MORE MEETINGS

In the spring it was time again for another social services meeting. At this meeting the home based nursing team suggested that Ben should be assessed for NHS continuing care funding. They explained that if Ben was granted this his homecare would be taken over by a team of nurses but that it was such a large team that he may not even see the same person once a week.

We were very against this as we knew it would not be safe for Ben to be cared by a large team of people. But regardless of what we said the nursing team and social services began to talk about us and plan what would happen as if we were not even there.

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After the meeting I phoned the home based nursing team and told them that they did not have our permission to refer us for continuing care assessment. We knew however that now social services knew that this may be possible that they would push us to do it and cut our hours if we didn't.

We became very worried again, once again the running of our home and care of our children was being taken out of our hands by people who simply didn't have any idea how this would affect us all, didn't take into consideration what we said or seem to believe it and I am sure did not care. I was scared Ben would not be safe.

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MORE TREATMENTS

I had saved up for Ben to have some private physiotherapy assessment. There are lots of different methods and theories for encouraging the physical development of children with brain affected disabilities. I wanted a second opinion and to ensure that we were not missing any tricks.

Ben enjoyed about 6 sessions with a different physiotherapist who confirmed that we were providing Ben with all the necessary stretches to help maintain his muscles but also showed us some different ways we could work to provide him with variety.

Ben was getting symptoms of urine retention, often whole days went by and he did not pass urine and occasionally a whole night would go by when he didn't despite us putting over a litre of fluid a day into him. I turned again to the canavan web board and found that some other children had this symptom and took some medication for it.

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I talked to our doctors about it but they did not seem convinced. So we started keeping records of how much Ben's nappies weighted, we already kept records of all fluid input. I could then demonstrate that some days Ben had fluid retention. He was started on the drug Doxazosin to improve his symptoms and had the first dose of this in hospital to ensure there were no adverse affects.

I also put a post on the canavan web board asking about Botox in the arms and legs of children with canavan disease. To my surprise a family in the UK responded by saying that their child had had successful botox in all four limbs performed by a doctor who was based in Scotland.

 

BOTTOX

I had been increasingly thinking that Ben needed botox to maintain his ability to do the physical things that he was able to do and thus maintain his quality of life, despite him being previously denied this locally. I spoke to Ben's paediatrician about this and somehow he actually knew the doctor in Scotland who could perform this and later in the year the doctor was due to come to Norfolk.

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It was arranged that the doctor would see Ben when he was in the area, thanks to our paediatrician. The doctor was very experienced with using botox and was quickly able to identify where to put the botox in Ben's limbs. Ben had 16 injections in total under sedation, we were told he would take about a week to start to see the full affect.

Just four days later Ben attended musical keys for his usual weekly group. Sitting on the floor with him on my lap joining in the actions to the songs I felt that I had my little boy back, Ben had been getting increasingly stiff over the last 18 months and suddenly we were able to move again. I was so pleased for him and again thankful for the contact of the canavan web board that had again benefitted Ben.

Overall we had a difficult summer, one member of our care team was on maternity leave and one member was off with a long term injury. We did everything possible to try and fill the vacancies without success. I didn't know but when social services grant you x many hours direct payments a week if you are unable to find the staff they are obliged to help so they eventually turned to care agencies to provide our staff.

It took a few months with many difficulties to find the right agency with the right staff but finally we built up a small care team for Ben.

 

BUGGED BY BUGGY

Ben had well and truly outgrown his special buggy. Speaking to a friend whose child had the same buggy in a bigger size she happened to mention that her child was getting a new buggy, I said that Ben needed a new one and she said she was happy for us to have their bigger one once it had been replaced with a new one.

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We were referred again to wheelchair services but it turns out that they cannot allow used buggies to be issued to other families, I was told that once the buggies of this type were used and outgrown they were put in a skip! I couldn't believe it the buggies were still around the £2000 mark and now the NHS only gave you £500 towards one so the rest of the money had to be raised by families or applied to a charity to help fund only to have the buggy thrown away.

As my friend had purchased her buggy herself she was allowed to give it to me but I was still left shocked by this waste, we advertised our own outgrown buggy and some other equipment we had outgrown and passed them onto other families who could use them.

Before I had a special needs child if I had thought about it I would have assumed that the NHS provides essential equipment such as wheelchairs and standing/walking frames to all people, especially children who need them, but it does not.

Through one of the disability newsletters that we receive I read about a speech therapy voucher scheme run by the charity Cerebra, we applied to the scheme and we are very grateful that Ben was awarded a £500 voucher towards speech therapy which we chose to spend with a local private therapist that had been recommended to us by several people.

This therapist is great she has enthusiasm to match Ben's and ours. She said to me "this is a little boy who is trapped in a body that does not work" she was right and we were thankful that for once someone could see it. She referred Ben to have his adenoids looked at due to his noisy breathing.

 

IN CONTROL

With the application for the speech therapy scheme we were also sent a Cerebra news letter. In this letter was an article about a scheme called In Control. I read the article which featured an interview of a family who had set up their own care package as I read the words that the Mum of one family said I actually gasped here was someone else going through exactly what we were. I went to the In Control website and read more stories.

The thing I was most amazed to find is that it is possible and legal for social services and the NHS to combine their funding and give a person one care package. This is something I had once asked for and been told was not legal, another lie.

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The family most similar to ours had written to their local NHS children's services commissioner so I decided I would to. I found out the name and address of the relevant person in our area. I explained our situation. I asked if they would consider a package of care for Ben that was not the norm but what he actually needed. To my relief a reply to my letter came that said they would. With this information we now went ahead and asked for Ben to be assessed for NHS continuing care.

 

TAKING CONTROL

I realised that all of Ben's life I had been talking to all of his professionals about the difficulties of looking after him and maintaining any kind of family life, but time was ticking by, my children were all growing and nothing had been done to improve things. Inspired by the In Control website I sat and wrote down what I really wanted and then I set about achieving as much of it as possible.

I talked about the problems and wishes for improvement with Adrian. We decided to get Ben his own phone for all of his professional's phone calls, there could easily be as many as 30 phone calls a day into our house for Ben. Our health visitor wrote to everyone with the new number asking them to use it, not everyone did at first but we persevered. Now I keep this phone on week days 9-5 and turn it off after that. I know that if this phone rings it's someone who needs me to stop what I am doing and talk to them properly and if I can't I now ring them back as soon as I can.

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I started trying to keep one day a week free of appointments and one week a month free of appointments to allow for some normal family time together, this wasn't always possible but it was an improvement.

I asked if a couple of Ben's professionals could be changed, I find this really hard to do and I don't want to hurt anyone’s feelings but I feel it is important that we are surrounded by professionals who are on our wave length. I don't expect everyone to always agree with us but I want to feel that the people that are helping us care for our son understand us and his needs and that I am not fighting against them or justifying all the time decisions that I make about my child.

Our health visitor retired, this was a blow because she had been with us for five years and had been a really good source of support and information. Since then (just over a year ago now) our health visitor has been replaced another four times due to changes in our area and I think the area that they now have to cover being too large.

Ben was granted NHS continuing care status when his assessment came to the panel.

Another Christmas came around, we decided to quarantine ourselves as much as possible for a couple of weeks before hand and for once we were all well on Christmas day. What’s more we had a carer come in for a couple of hours on Christmas day to help us all get on our feet in the morning which was a big help.

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Chapter 7

TOES

Ben had continued to have recurrent swollen big toenails since the day of his blessing service when he was 18months old; he was now 6 years old. We had seen our GP and health visitor numerous time and tried creams without success. We had seen a chiropodist who had not been able to help although we had followed his advice on foot care.

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During an appointment with one of our other consultants it was suggested that we meet a new paediatric orthopaedic surgeon in our area, this doctor as it turned out had previously worked in a hospital especially looking after children with cerebral palsy. She recognised that it was the angle at which Ben's foot was growing due to the stiffness that is causing his big toe nail to grow into the fleshy part of his toe. She was also prepared to perform Ben's future botox.

So Ben had both of his big toenails removed and in the same operation he had his adenoids removed as they had been assessed by now and were very large. Ben was in hospital just 3 days and recovered well. The district nurse dressed his toes for him at home for three weeks and he only had to go back to hospital for the first dressing. I was shocked how bad Ben's toes looked when he had his first dressing removed but the toes healed quickly and now cause him no pain.

 

HOLIDAY

We really wanted to have a family holiday so we hired a lodge in Yorkshire in May and took a carer with us for a bit of help each day. Ben was now 6, Harriet 4 and Sam 1. It was hard work but we had a good time. We also achieved one of the things I had always hoped to do with Ben, take him up onto Ilkley Moor, we didn't see any ducks flying backwards, just lots of rain!

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After an assessment a new standing frame was ordered for Ben, it was great to see him standing again and he enjoyed doing so, he looks so tall now. A really helpful thing about being part of continuing care is that if Ben needs a piece of equipment the funding is agreed much more quickly than when it was going through social services.

Later in the summer we booked our carers to come and stay with Ben at home for a weekend and we took Sam and Harriet to the seaside just 1/2 an hour’s drive away for the weekend. We let them spend a long time digging in the sand and playing in the sea and doing some things that it is less practical to do with Ben.

 

TREATING MUM

I think since I had had Ben that I have had untreated post natal depression. I don't think this explains my reactions to or excuses the way that I have been treated however. I think I had mild post natal depression but was made dangerously ill by the lack of support and way I was treated mainly by social services but also by others close to us professional or otherwise.

Since I struggled when Harriet was a baby if the carer was ill or there was a conflict with one of Ben's professionals or worse still the carer I felt myself sinking. Prior to and particularly the few days after our social services review meetings I would feel as if I were shaking on the inside and cry all the time. I found the amount of professional people that I had to justify all the little, even personal decisions I make about my child very difficult, but actually who wouldn't?

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When I wake up in the morning I feel that I am already at work because a carer is only a few minutes away and although I am grateful for this and need the help I wish I didn't, we have to act in a professional way in our home all the time when a carer is present, you can't pass wind or scratch your bum! But that's our life mostly I can handle it, all people have bad days, but some days I felt really bad as if the darkness was surrounding me and I couldn't even think, struggling to make the simplest decision like what to make for dinner or even have a conversation.

The panic attacks were the worst, I found these really hard to deal with. It would mostly happen when I was away from home, perhaps if Adrian and I got the chance to go into the city alone occasionally or if I had gone out by myself perhaps for the short drive to pick up some medicine. I would feel like I was shaking, like I wasn't safe, I couldn't explain it or describe it, I struggled not to cry. This might especially happen if I saw a young child that reminded me of our own. When I am at home, busy, just dealing with day to day life I don't worry so much but when I am away I start to grieve, I stop being too busy to notice how much I am hurting. But sometimes the feelings of shaking and being unsafe would happen when I was at home.

But on the outside it wasn't obvious that I was upset, it appeared that I was moody and in my state of confusion I would snap at Adrian or the children, often even shouting.

Many times I spoke to the GP, social worker and health visitor quite frankly about this, all the things you are supposed to do. I was referred to see a counsellor for a while but this was really hard to fit in and although healthy didn't relieve the cause of the symptoms. I felt terrible without warning far too often, so terrible that I knew it was not normal.

Earlier this year I went back to see my GP and insisted something was wrong. She gave me a questionnaire to fill in, this was really helpful because it asked me questions about my symptoms that I had failed to describe to her previously. My GP is excellent but I don't think she had realised how bad things had been. So she started me on a course of anti-depressants. The medicine I am taking is not addictive and they are not happy pills, they don't make me feel good when I am not. It is just the kind of medicine that re-balances the chemicals in the brain, the ones that can get all muddled up when you have had a baby.

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I did suffer from headaches and queasiness for the first 2-3 weeks but this passed. 6 months on I am about to start reducing the dose and come off the medicine. I don't feel high, I still get bad days, I still get upset when there's a reason to be, I still get PMT. But I don't get panic attacks or feel like I can't go on another minuet for no reason at all. I feel like me, I had almost forgotten me, I'm so glad to be back.

There's still such a stigma about depression but I was ill so I took some medicine and now I feel better, it's as simple as that.

 

JEJUNAL FEEDING

Ben's reflux seemed to have been back for some time with lots of gagging. We asked for a second opinion and our local surgeons put their heads together. It was decided that we should try fitting Ben with a jejunal feeding tube to see if jejunal feeding offered him some improvement. This should work in theory because the food would be going into Ben's system lower down and therefore less able to come up and give him reflux.

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However because we know that Ben does things in his own way and does not always want to work to our plan we opted first to have his gastrostomy button changed to a gastro-jejunal feeding tube which meant that he didn't need any actual surgery, just a trip to theatre to have the tube correctly placed. Happily over the summer and autumn Ben has had a good weight gain and we have managed to get more feed into his system than before. The downside of a gastro-jejunal tube is that if it needs to be replaced unlike a plain gastrostomy button Ben has to have a trip to theatre to ensure that the tube gets placed correctly all the way down in his jejunum.

After just five weeks the balloon that holds the tube in place burst and Ben had to have a second trip to theatre to have it replaced. After a couple of months we could tell that feeding this way was going really well and Ben was gaining weight so we asked for him to go onto the waiting list to have a permanent tube placed, although this would mean an operation for Ben overall it should mean less trips to theatre and anaesthetic because a permanent tube is held in place with a plastic disc rather than a balloon so the tube should last for two years.

Ben had his third lot of botox in the autumn, he seems to need this every 6 months and it is still working really well. It is not now possible for Ben to completely straighten his arms and legs but he almost can, we have been given additional advice by Ben's physiotherapist this year to ensure we are doing all we can to stretch and maintain his muscles and posture.

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Over the summer swine flu had been a big worry, in our household we had caught two flu type viruses in 3 months but we were not tested for swine flu so could not be sure that we had had it, fortunately Ben had recovered well from these viruses. We had a lot of staff sickness meaning we went several weeks without a break. I still find it really important to have just a few hours break once a week I find I feel much more able to cope and am a better Mummy for it. Finally in November swine flu inoculations were available and we all received a jab.

Winter again and Ben was admitted to hospital for his jejunostomy. The gastro-jejunal button was changed back to a plain gastrostomy button and a new hole was made a bit further down for the jejunal tube to be permanently placed. It was hoped that the surgery could be done by keyhole but when Ben was in theatre it was not possible for the surgeons to accurately identify the correct piece of his bowel this way so a small incision had to be made about 1 1/2 inches long. Because Ben had a larger wound than anticipated then he was given an epidural anaesthetic for a few days to keep him comfortable.

Ben left hospital after one weeks and is now in the process of his system settling down again.

 

 

AND NOW.....

Its 14 months since Ben was given continuing care status. In the mean time we have stopped using the home based nursing team. I hope we are almost there, we have been working towards finding a company to run a care package specifically to meet Ben's needs with a small care team trained in Ben's care needs. The continuing care department have been slow to respond and seem under-staffed but at least they talk to us as if we are human.

Ben is recovering well from his jejunostomy and we are hopeful that we are moving on to a period of improved feeding and well being.

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It's nearly Christmas 2009. Last December I was really stressed and seemed like I was chasing my tail so this year I decided to get everything done before the 1st of December. I love Christmas so this year by the last week of November I had almost everything wrapped, cards written, crackers made so now we can enjoy the rest of December. It's Harriet's first Christmas at school, now she and Ben are at the same school we are looking forward to seeing them together in all the school's festivities. Sam is coming up to his second birthday in just a few days time. I have even joined the community choir at the children's primary school which assembles each year to support the children in their Christmas production.

And so life goes on. Whenever I think I have everything sorted out and I know all there is to know to live as best we can within the system of a special needs life something happens and we have to get over another hurdle. Just when I think Ben is stable and there's a quiet period he does something that will test us. So we will keep learning, adapting and never give up. We have our ups and down like anyone.

When I think about it am a person whose body does not find it easy to carry a baby full term and who carries the gene for canavan disease, but I have a fantastic a husband as it's humanly possible to be and three amazing children, it's just that one of them does have canavan disease and that's not something I had planned.

When I think of the things I had wished for when I was young, the things that really matter then I know that I have everything that I ever really wanted and I think I'm pretty lucky to be able to say that. I don't know what the future will hold, how long we will all be together, I don't think of what will come, at least not if I can help it. All I can do is keep making memories, keep trying to focus on the things that are really important, keep trying to be the best Mum that I can be, because that's the best that anyone can do.

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Ben with his tulips