In, out, in , out..... (poorly again )

Tuesday evening Hope was not a happy Honkey. Over a couple of hours her unhappiness increased. She became pale. Then she was sick. She's only been sick twice, both times a sign of an infection. Once being an ambulance job. So when she threw up again, and was becoming more and more distressed, we took her to hospital. I know that might seem an over reaction. I'd not rush dolly and moo to hospital if they'd been sick, but Hope is so fragile, and I'd rather we took her, prepared, than wait a few hours and need an ambulance.

Hope has 'open access' to the children's unit. She is considered by them as a 'frequent flyer'. Our favourite ward doctor G was there to meet us. She knows Hope well. She's also the only doctor that can get a line in Hope quickly. She did a great Job getting it in first time!

Hope's obs were fine. Sats 100, temp 35 (Normal for Hope). Her blood gases were fine. And her Lactate (our main marker) was good- 2.0. Hope was calmer and I felt reassured. So we discussed with G, the fact that she most likely is ok, and that the other blood labs wouldn't be back for a few hours, so it was agreed we could go home, with the canula in, and the community nurse would visit in the morning, and in the unlikely event that there were any issues with the other lab results, she would call us.

6 am the doctor rang to say her urea was sky high-11 (should be less than 6) so we were to go back in for fluids and follow up bloods.

Hope slept through the night. Woke for about half hour, then slept some more.

I managed to get about 150ml of dioralyte into her, and by the time we were back in, she had perked up a little bit. Her other bloods were normal, no sign of infection.
We were lucky that R heard she was on the ward and came up to see her. He said if her next urea came back above 8, she'd have to go on IV fluids for at least 24 hours. To my amazement it came down to 7.2 so we managed another lucky escape.

Though once home again I questioned whether we really should be. She wasn't her usual happy self. And in fact mainly slept and slept. But I've learnt from other families with PDCD kids that they all tend to sleep lots when they are poorly.

Today (Friday) started off with a nasty BIG seizure, but we rode it out, and thankfully by the five minute mark chris and I could tell it was going to pass. She slept another couple of hours, but by midday, was eating and drinking and rolling and very chatty. It's now 23.47, and she's just going off to sleep. That's more normal :0)
All just in time for her big sister's 12th birthday in the morning!

Spec-taculor!

At her last opthamology appointment, it was felt that her visual awareness (Hope has cortical visual impairment) had improved greatly, so where she's always been long sighted, and previously they felt glasses wouldn't make much difference, now they wanted to give her the best opportunity to see.

According to the man in specsavers, it was 'quite a prescription' and they had to order the lenses in. We chose some little miss sunshine (perfect) frames, and picked them up a few days later.

As predicted by the optician, results with such strong prescriptions can be amazing. We envisioned Having to build up the time she spent wearing them, but once they were on, she just began looking around. We only take them off her to go to bed and have a bath, and then we feel like we are depriving her of her vision. Ok, so I know her vision is still far from perfect, but they've definitely made a difference. And they suit her so much. I think some people Really suit glasses, and Hope is definitely one of those people. She already looks odd without them.

(name of post attributed to Rachel Bass who commented 'spec-taculor' on a pic of Hope with her new glasses :0). )

Desperately seeking keto

So knowing what we now know. The ketogenic diet that I've always wanted to try for Hope is one of the first courses of action. Unfortunately there's a 3 month waiting list in Oxford.

But knowing what we now know, I can't continue to feed Hope foods that are quite literally doing her harm :0(

I called our local dietician and explained, and said that I know she can't help us with the ketogenic diet (it's complicated and requires a hospital stay, starvation and very a careful monitoring in hospital, then lots of monitoring afterwards), but I can't continue to feed Hope the way we have been, so I'm going to start restricting carbs, and would really like to start the modified Atkins diet or a low GI diet (both of which can be started prior to the ketogenic diet if something needs doing ASAP, and don't require a hospital stay and aren't anywhere near as complicated. I asked for her advice and support. She declined!!! She doesn't 'believe in those type of diets for children'. Which is all very well... But Hope NEEDS a diet like this to maintain her health. I'm so angry at this dietician and I Hope our paths don't cross in the near future.

In desperation in turned to the Internet. Not even sure now what I was looking for.. Or how I came across it.. But I struck gold!! The Daisy Garland- a charity set up in memory of a little girl called Daisy, who had Dravet's syndrome, and died aged 6 from SUDEP (sudden unexpected death in epilepsy patients).In tears from reading Daisy's story, and my own distress, I called the number, not even really sure if or how They could help. It was Daisy's mummy who answered my call. Through my blubbering I think she understood my desperation, and said they might be able to help. The charity funds ketogenic dieticians in several hospitals. She felt that if we couldn't be seen by our local ketogenic centre (Oxford), we should be able to be seen sooner by one of their dieticians. Possibly in Derby or Nottingham, or maybe even Bristol. All I'd need is a referral from Hope's consultant.

......... (2 weeks later)................

Simple? Except this is the NHS we are taking about. All to do with funding. Swindon would rather we be seen by their partner hospital Oxford. Oxford were seeing what they could do- if Hope could be seen as an 'urgent' case. 2 weeks later I called (yesterday) to be told that Oxford has a 3 month waiting list, and to refer us to the Evelina @ guys hospital London. All this time waiting for people to refer.... Is time being wasted for my precious Honk, who needs to start this diet NOW.

The Evelina is far from convenient, London is not easy, but I would travel to the end of the Earth. But now, and we still haven't even been referred there!

The Oxford dietician rang today to tell me what I already knew (from chasing up yesterday). She said she hadn't realised the urgency of the situation and that I'd been waiting so long, but she does already have 10 children on her caseload (that's her maximum) and a long waiting list.

She told me to ring Swindon and get them to explain the urgency of the situation to Bristol. So I did. Straight away. To be told that the referral needs to come from Oxford. I think what this boils Down to, is whoever refers her, has to fund her. Different hospitals have pots, and no one seems to want to dip in their pot to get hope funded elsewhere.

So now I'm waiting again. I hope that the Oxford dietician has taken this case more seriously now. She seemed surprised by how much I knew. she was going to speak to her line manager... I don't know why. I'm hoping they are reconsidering. I hope she doesn't keep me waiting too long. I hate this waiting :0(

I have received continued support from Daisy's lovely mummy. She was kind enough to send me a DVD of a film made about the keto diet 'first do no harm', and some lovely daisy coin key rings, plus lots of information regarding the diet. She's also kindly offered to fund the gram scales (an essential item for the diet), and also a SATs monitor for Hope if I want one. As she pointed out, if she'd had one, her precious Daisy would still be here today. So if anyone is feeling charitable, and has a few pounds to spare, this really is a worthy charity. Or you can even donate without it costing you. By going through their site to amazon, amazon will donate to the Daisy Garland without it costing you a penny :0)

http://www.thedaisygarland.org.uk/

Xxx

Diagnosis!!!!!

It was back in June Hope had the muscle biopsy, I'd almost given up asking if there were any results, and was happy to bury my head in the sand. Certainly for the remainder of this year. Get Xmas out of the way. But on Wednesday, I opened a letter, addressed to Hope's consultant R, detailing the results and asking for him to break the news. No need since we seem to have unintentionally have been copied into the letter . Oh well

Anyway, it's not good. And that's a serious understatement , but I'm writing this update quickly whilst I'm not feeling too heavy hearted. She has a neurodegenerative mitochondrial disorder. The same one that I've mentioned so many times. The one I feared. The one that I was led to believe had been ruled out already. But the one that she just ticked ALL the boxes to:
Pyruvate dehydrogenase complex deficiency (PDCD). It's bleak. But she's here. She's doing ok. There's no cure. But 'therapeutic' options have been suggested.

Ironically the main one being the ketogenic diet! The same diet I've been asking her consultant about since she was one. The same diet, that after constant nagging on my part, her consultant finally agreed to referring us to the specialist in Oxford to discuss (his reluctance was based on how well hope has been doing without it). Fortunately I'm
Well armed with knowledge, I've bought the 5th edition of the ketogenic diet medical book and read and highlighted already.
Unfortunately my curiosity over the last 2 years has led me know too much about this disorder. But already I've gained a bit more positivity from a support group for parents of PDCD kids. I'm inspired by a little girl L and her mum. L is 9!
Her mum has fought hard to keep her. So will I!

She's called Hope for a reason. I won't let go!!!!!! I can't :"(

Acidotic

Acidotic

After her clean bill of health on the Friday, we had a few lovely days camping in weymouth. Hope was happy and well apart from teething a little. After being home for 2 days on the Wednesday  we were just having a quiet day catching up on washing. About 11am, Hope started screaming. We gave her calpol and she remained inconsolable. Her feet were twitching like they were after the pool fiasco. An hour later, I rang the childrens unit to say I was bringing her in to be checked over. I told them I couldn't put my finger on what was wrong, but something was. I was hoping that we'd be sent away, having over-reacted. I was wrong. She was acidotic!!! Her blood was too acidic. And dehydrated. So she was admitted again. She was on iv fluids for 24 hours and was still too dry so they upped the fluids. 

The second morning, the consultant on the ward rounds was dr Z. I've mentioned him before. He used to be her consultant. I thought about refusing to see him.  He recognised Her, but obviously couldn't remember her from over 2 years ago. So when he said 'haven't I seen you before?' I was able to get something well overdue off my chest. 'yes' I replied. 'you were her consultant, and when she began fitting at 3 months old, you didn't believe me. I kept asking for an EEG, and you told me you thought she had colic. As it turns out, by the time she was seem by Ravi, she was in a constant state of epilepsy!' all that said in front of 2 SHOs and a nurse. remember us now? I'm sure you'll not make that mistake again! And I felt better for saying it :0) naughty me I know. But I do tend to hold grudges! 

Ravi came to see Hope later that day, ....

Again I didn't finish this post and now can't even remember what Ravi  said. What I can remember is we went home. Then yo-yo-ed back in and out a further 2 times. Apart from giving her IVs, there's not a lot that can be done for these new problems. Her blood work is all over the place: high urea, teetering on and off being acidic,high platelets... And a baffled consultant. So along with the previous aspiration pneumonia, and now the increasing need for extra fluids, the ball is rolling for a gastrostomy (feeding tube into the tummy).
And this last part broke my heart .. .Ravi's words 'this may just be the next stage of things :0(

We were discharged knowing her blood work wasn't as it should be, but knowing that she cannot spend her life in hospital in IVs.

She seems ok. Most of the time. I think. But it's nerve racking! We now have a hospital bag packed. Ready to go if we should need to. But touch woos, since I packed it, we've not needed it. We've actually done 9 days now without bring admitted. She's back in on wednesday for a check up, to see what her bloods are doing a rest dose of bucal midazolam (since she reacted so badly to the rectal diazepam)

Not the best summer! Far from it! 

The 'P' word

The P word (aspiration pneumonia)

In my last post I did really skim over the fact that Hope had had a diagnostic skin and muscle biopsy, and following that, had been hospitalised with a nasty chest infection. It was only after she was better that the P word was mentioned. That it might have been a lucky scrape with it. I was glad that I didn't know, that that's what they were thinking!  So after 3 days in hospital, IV fluids and anti biotics, Hope was well and home again. We had a week of our usual busy schedule off therapies etc, but I decided she was well enough, and would love to go for a Honkey splash at the hydrotherapy pool session with her other special friends. Hope absolutely LOVES swimming! She goes crazy!!! And has recently even began to 'swim' (staying afloat and moving through the water in her own style). 
But half hour into the lesson, hope became distressed. This NEVER happens when she's swimming. I took her to the side of the pool to calm her and see what was wrong, and then I can't really explain what happened cause it all happened so fast...but she kinda went limp and changed colour. I knew something was wrong so got out fast! Then she was fitting (? but not her normal type- lots of twitching in her legs and her feet were going crazy, and during this hope was screaming and looking at me to help. It was awful!!!

And my nightmare was made worse by the fact that on this occasion, stupidly, I'd left my mobile and her rectal diazepam (epilepsy rescue medicine) in the car. So I had to quickly get dressed (I didn't dry myself!), so I could get help. As time went on, I realised I probably shouldn't even give her the rectal diazepam as this was a different kind of fit, and also because of her change of colour. So the school first aider called for an ambulance. Hope vomited (something else that's very rare for her- last time she did was a 999 job too). By the time the ambulance arrived, the nasty fit seemed to be over, but she was still very distressed and twitchy (with most likely left over epileptic activity). So off we went in the ambulance. 

Hope was still very distressed once we arrived, so was taken to the paediatric  resuscitation bay (not that she needed it at the time, just they have more than the basic equipment there). So she was wired up to heart monitors, sats machine etc. 

Her lactate level was 6.8- ridiculously high, and another honkey record breaker. Bearing in mind the chest infection the week before, she had a chest xray done in resus, which confirmed pneumonia. So the doctors planned out the course if treatment; IV fluids and antibiotics and arranged for her to go up to the ward. But whilst waiting for the arrangements to be made, Hope began fitting again. Nothing spectacular, not like earlier, but strong jerks, so the doctor decided after three and a half minutes to treat with rectal diazepam. As before (Hope's only had it in hospital, this being just the second time) Hope initially seemed to be quite enjoying it's effects and looked high as a kite!!! The fit still lasted about ten minutes, then she rolled around spaced out for another ten minutes or so, then she zonked! Proper sparko fast asleep! Then her Sats dropped!! So she needed oxygen to bring them back up. But then they dropped, and continued dropping even with the oxygen! Scary scary stuff watching the numbers going down and the docs looking to eachother, moving around quickly and efficiently. Then they were giving her an airway and  manually bagging her. The help having her airway open seemed to do the trick and eventually she was able to Cope on oxygen. So this postponed our trip to the ward for several hours, and when we eventually made it up 5 or so hours after we first arrived, it took a team of doctors and a  nurse to get her and all the monitoring equipment up to the ward.

The next few days were really a blur of stress with just being there in hospital, stress with canulas that kept not working, then doctors struggling to get new ones in, 12 hourly bloods to monitor her lactate, infection rates etc.  

I've lost track of days, but I'm certain she was admitted on the Friday, and let home on the Tuesday evening ( though not discharged as she was to return daily for IV and IM (intramuscular) antibiotics and bloods. 

We were told that the pneumonia was mostly likely caused by the general anaesthetic for her skin and muscle biopsy. The theory being that the GA caused muscle weakness, leading to Hope aspirating on food/drink, leading to the infection, and building up to 'aspiration pneumonia'. It's really hard to accept this, though I know it's right. She was fine previously. Pretty much. Though in hindsight she had given me a scare a few weeks earlier with her breathing after swimming. 

Though the infection was clearing up, this was thought to be a 'chronic' as in a long term condition, that may take weeks/months to shift. Possibly leaving her lungs, in a new, not so great condition. So before we left, I was trained to do chest physio, to try to shift some of the secretions, shown how to suction her secretions with a machine (that we now have one of at home). Because of her issues with her hydration and lactate, it was agreed that Hope needed extra fluids via an NG tube, so we were also trained on that. 
She was also prescribed a 'patch' to wear behind her ear to reduce the secretions. 
I was heavy hearted about the chronic condition, and really feeling like we were on a very slippery slope. 
Though very glad to be going home after her longest  admission. And just in time for Dolly and Max's sports day. 

(just realised I never got round to completing this post, so will gloss over whAt I can remember- it's been a mad few weeks. And not in a good way!)

We made sports day. The next day she went back in for bloods and got admitted for high lactAte again. Then diScharged a day later.
Our life for the following two weeks ish, evolved around giving meds, tons of fluid via the NG tube, dealing with NG tube problems such as it getting blocked, Hope coughing it up etc, along with every few days having blood tests to check her CRP (infection rate) , platelets (which were far too high) and lactAte to name but a few. I've become quite the expert now. And we feel too much at home at the hospitAl.  We also came under the community nursing team (as lovely as they all are, I saw this as a step in the wrong direction for us), and took delivery of and received training on a suction machine. 
The patch dried up Her secretions on her chest (but they increased her seizures so we had to stop using them) her lactate settled (or rather we settled with a 4 as being good enough), her platelets were slowly coming down, and her CRP was less than 1. She'd won! She bloody well fought off nasty ol' pneumonia, and she beat it! And as for it being chronic.. And me trying to come to terms with Hope's new chest state and noises... A miracle must have occurred, because 3 weeks from the pool episode, Ravi (her amazing consultant) listened to her perfectly clear chest, and based on this final set of bloods, gave us a clear bill of health. As clear as we are ever going to get anyway ! WAY TO GO HONKEY!!! <3 
Xxx 

Hope- a snapshot

There's lots I could and should update about the last couple of weeks, but just thinking about it makes me tired. In short, Hope has had a skin and muscle biopsy in the hope we get get some answers (or not) about her condition. Shortly after this Hope was very poorly and was admitted to hospital with very high lactate (5.8 :""(  ) it seems she's been aspirating and aquired a chest infection, though at one point suspected pnuemonia! She needed IV fluids, antibiotics and even oxygen (she's never needed that before). She's home now, but not her usual happy self and I'm sad and worried about her.

Anyway, unbelievibly (to me lol) she's due to start nursery in September. So I've been finishing off my parental contribution to her statement this evening, and I thought it provided a nice snapshot of where Hope is upto these days. It also perhaps shows me as a neurotic, fussy, overprotective, over anxious parent. Oh well :O)

My Child's early years

We were aware that Hope may have some kind of disability, before she was born, based on brain abnormalities picked up on scans. She spent a short time in special care due to her low birth weight and for observations to be made.

Hope left hospital at one week old, and seemed to be developing 'normally' until she began having fits. This was initially misunderstood by her consultant, and by the time epilepsy was diagnosed, Hope was in a constant state of epileptic activity. She'd lost all previously gained motor skills, all of her little personality and even her sight.

Hope began epilepsy medication, and though this gained some control over the fits, the medication left Hope very drowsy for a long time. Hope is much more accustomed to the medication now, and gradually overtime her personality came back. She still has seizures most days, but they are much better controlled. A lumbar puncture carried out at 6 months of age, suggested that Hope may have a mitochondrial disorder. Hope is now being seen at the John Radcliffe and we are awaiting results from a skin and muscle biopsy.

Though I'm aware that Hope is extremely behind in her development, she never ceases to amaze me. She works very hard to gain new skills, and each little new thing she learns is a huge triumph for us both.

Hope is seen and supported by many. These include her consultant, Dr Chinthapalli, her epilepsy nurse, her physiotherapist, occupational therapists, her portage teacher and koala’s key worker.
Hope has been attending koalas since she was less than one year old (I am sorry I can’t recall), and having portage at home since March 2010.

What is your child like now?

General health

Hope has epilepsy that is kept quite well controlled by vigabatrin. She's currently on 900mg twice daily (given in yogurt) but this dosage is based on her weight and seizure activity, so changes regularly. Hope also has a rescue remedy of rectal diazepam.

She's generally quite well, though recently she's had a succession of chest/throat infections. Hope can become dehydrated (she's not a big fan of drinking, and even less so if she's poorly) and due to the nature of her possible mitochondrial disorder (high lactate) it's important for her to be well hydrated. Therefore it’s important to observe her fluid intake, plus watch out for signs of dehydration. And if it's suspected she needs to be admitted to hospital for IV fluids.

Hope does suffer with constipation. She is currently having half a sachet of movicol in the morning. But we also use a pessary if necessary.

Most recently, Hope has undergone a general anaesthetic for a skin and muscle biopsy (on her leg) at the John Radcliffe. We hope that the results from this may shed a little more light on Hope's condition.
Following this surgery, Hope was admitted to the GWH. She'd remained very sleepy and had been aspirating and her lactate levels had become very high.  She needed Oxygen, IV fluids and antibiotics.

Physical skills
Hope can roll, though sometimes gets an arm stuck. She can lie on her tummy and raise her head for a few minutes at a time (if she’s in a good mood and has something she likes to distract her). She has good head control. She is now able to sit by putting weight through her arms, though she's still quite wobbly. She can on a good day sit for a couple of minutes, though lacks the skills to save herself when falling. However, recently I've witnessed that she's making an attempt to.
With the support of a person, and/or a therapy ball, plus her splints Hope can stand.

Hope can grasp objects of interest to her, and will pass from her left hand to her right.
She uses both hands to operate switch adapted toys/ communication devices.

Self help
Hope needs to be dressed and undressed. She will attempt to help by pulling or pushing her arms through sleeves.

Hope needs to be fed. She manages a mashed consistency of food, and can also eat certain whole foods eg. sandwiches, soft fruits etc

Hope wears Nappies.

Hope doesn't mind her teeth being brushed. I count very slowly from 1-10 for the bottom teeth, then repeat on the top teeth.

Hope has very curly, difficult to brush hair. It's best to tease knots out which she's in the bath and has conditioner on.

Hope needs to have her face and body washed, and also her nose to be wiped. Counting slowly to ten will reduce her stress.

Communication
Hope has a repertoire of 5-6 words. Sometimes she uses them appropriately and consistently, but not always.
Hope is a very sociable person, and is attentive when being spoken to. She will turn take and make vocalisations, and very occasionally imitate. Now that Hope's vision is improving, she will sometimes make eye contact, though often this is brief.
I believe Hope understands a few words. She's able to make choices of people, favourite toys and favourite books. Given choices, hope will give a clear response, either by a vocalisation, or a very big clear smile
Hope is able to express her desire to have a change of position/scenery/activity by saying 'OU..' (out). This is made clearer by her use of body language, whereby she will kind of arch her back ready to be lifted.

Playing and learning
Hope's favourite toys are: story time bear, Elmo, pom poms, crinkly foil and fluffies (soft material). She also enjoys playing with her switch activated toys.
She doesn't really play with other children, but loves being played with by her siblings. She enjoys being tickled and also enjoys wrestling with her brother.

Hope has a few stories that she really enjoys. These tend to be books that have noisy buttons, or are feely books.


Hope enjoys going in swings and on bouncy castles.

Hope isn't occupied by television, but she does really enjoy listening to music, especially children’s songs and nursery rhymes. She loves being sung to. Especially rhymes that have actions e.g. ‘Tommy thumb’, or ‘round and round the garden.’  ‘Twinkle twinkle little star’ will calm her if she's upset.

Hopes all time favourite Activity is doing 'row row the boat'. She likes to sit on a lap and hold hands and rock forwards and backwards. Sometimes she will initiate this by rocking when on a lap, or by saying (what we think is) 'row'. Hope knows 7 verses to this song though, and will become frustrated if the person singing it doesn't do her full version.

 Relationships

Hope is very much a mummy's girl, as we are very rarely separated. If she's unhappy or poorly she needs a cuddle from mummy. She has a lovely relationship with her Daddy and enjoys playing, talking and relaxing together in the evenings.
Hope has a wonderful relationship with her siblings. She gets excited when they interact with her, and has a very special and unique relationship with each of them, each of them bringing out different aspects of her personality and different behaviours. Hope also has good relationships with her portage teacher, koala’s teacher and her rainbow trust support worker. She doesn't seem to be wary of strangers, but isn't as sociable with people she doesn't know. She tends to have a sense for therapists and doctors, of whom she's least happy with.

Behaviour at home

Hope is quite demanding in attention. She's not able to really entertain herself, so requires attention. She's used to having attention all the time, and becomes upset if she's left unattended. Hope has quite a short attention span (even with her favourite toys ten minutes maximum) and will show that she's bored by whinging. She doesn't like to stay in one place for long either, so moves between various chairs, laps, floor, standing frame etc.
Hope is very affectionate, and loves to be kissed and cuddled. She will really snuggle in for a cuddle if that's what she wants. If she's tired, she will become grumpy and need a cuddle and her dummy.

 At preschool/ nursery
Hope enjoys the time she spends at koalas. She has a lovely relationship
with her key worker, and isn't upset if I'm out of the room. Hope enjoys many activities here, including messy/sensory play, water play, and painting (with her hands and full adult support). She enjoys playing in the garden on the swings and trampoline. She really enjoys going into the sensory room and using the switch activated lights/sounds. Hope does interact with some of the children- usually when they are in a very close proximity.

My general views

How do you compare your child with others of the same age?

I am aware that Hope is very delayed, and much more like a young baby- though in different areas of development this varies as to how far off she actually is of her chronological age.

What is your child good at or what does she enjoy doing?

Hope is having lots of success with switch activated toys and communication devices, and I see this as a strength and something that I want to continue with, as she's enjoying the control, and I see that it has a massive potential for the future of her learning and communication.
Hope enjoys playing with these.

She enjoys stories, music and nursery rhymes. Hope also enjoys rolling around on the floor, and playing with crinkly foil, pom poms and fluffies.

Hope loves water! She loves having a bath or shower, and loves swimming. She becomes very happy and active in the water, and is now able to stay afloat and kick her legs when she has armbands on. She has achieved ducklings’ stage one in her hydrotherapy class.

What does your child worry about?

Hope's only worries as far as I'm aware, are being left on her own.

My worries

Hope being left unattended- even sleeping I don't leave her as she's most likely to have seizures upon waking.

I'm worried about Hope's epilepsy care plan, which I think needs reviewing, but my main concern is that maternal instinct tells me when rectal diazepam needs to be given or an ambulance called.

My biggest concern is Hope's seizures (which can be very subtle) going unnoticed. I need to know the length and regularity and strength of them, so I know if it needs treating, or her meds need increasing,

I'm worried about Hope sleeping too much at school- this can be a sign of high lactate

I'm worried about her aspirating, which is something she's only recently began doing

I'm worried about her feeding. I'm the only person that ever feeds Hope. I know how to feed her, how to place the food in her mouth, when it's getting stuck on her high palate and when it needs fishing out.

I'm worried that Hope will be unhappy in her school seating. She's been very fussy with postural seating, and she's going to have a new unfamiliar chair (still undecided). Also she doesn't like to spend much time in any position.

I'm sad that Hope will be unable to tell me what's happened at school each day.

I'm worried that all of the ways in which Hope communicates can't actually be translated onto paper, but can only be learnt by spending time with her and myself so that I can translate her needs.

I'm concerned about Hope's splints (orthotics), which she'd ideally be wearing to nursery. But we have ongoing issues with them, and she regularly manages to slip her heel out of position and this rubs and causes her discomfort. They need to be checked and put back on several times a day. Putting them on is a tricky procedure in itself!

I'm very concerned about leaving Hope. I didn't let her out of my sight until she was 18 months old. I've only ever left her with her Daddy, and her rainbow trust support worker, and yet it seems I'm expected in September to leave her with virtual strangers after just a couple of half hour visits. Which I won't be! I think going to nursery would benefit both Hope and myself, but this will happen, only if I feel comfortable with leaving her, knowing that the teacher and staff know her well enough.

My child’s special educational needs

I believe that Hope will need one to one support with someone who knows her well. She will need to have a structured individualised plan to take into account all of her needs; socially, emotionally, along with physiotherapy and developing fine motor skills, which will take into account her disabilities and her visual impairment. A clear epilepsy plan will need to be in place. Hope will also need appropriate seating that she is happy with.

I believe these needs can be best met by Brimble Hill School. I believe a slow phased transfer of knowledge about Hope (from me to the staff at the school) will allow for a smooth transition.

Therefore I’d like to have much more contact with the school and the staff before I feel ready to leave her there without me. I am pleased the school have agreed for her siblings to have a visit, so they can see why I have chosen this school over their school. However, I feel that the school are reluctant for me to spend much time there other than a couple of half hour sessions. As I have said previously, I will not be leaving Hope there until I feel   they know and understand her well enough. I am aware that Hope is not required to start her formal education for quite some years, and I am prepared to postpone this if necessary.

Dear Tesco II

Dear Tesco
After just leaving your store in tears, feeling frustrated and discriminated, I am writing to you today to inform you if my intention to contact both the local and national press.
Despite my pleas to you since November to provide a suitable trolley for my disabled daughter, and despite numerous members of your staff taking on board my requests and promising to deal with the situation (both in store and from customer services) I still find myself more than six months on struggling to carry out my shopping.

After giving up on in store staff who made promises, I wrote to yourselves at the start of may. My letter to yourselves can be seen on my daughters blog. Www.honkeyplonk.blogspot.com (2nd or 3rd post down). Upon receipt of this, I did receive a call from someone called Rachel (?) who apologised profusely, and assured me she was on the case, and that she'd call me back by the end of the week. I'm no longer surprised by the fact she never called back.

Your disregard for mine and my daughters needs, in my opinion amounts to discrimination. Fortunately for you, my daughter is going into hospital on Monday, so I am unable to get the press ball rolling, but I assure you, it will be at the top of my to do list upon her discharge (hopefully Tuesday or Wednesday).

Yours sincerely
Caroline Matthews

A demanding 2 year old in Cornwall :O)

Time for an update since I have a few hours on my hands on the homeward bound 
trip from Perranporth.

We've had a gorgeous week, and been blessed with beautiful weather. Perranporth 
is without doubt my favourite beach in the Uk. Shame it's a good four hours away 
(or 7 on the way here due to traffic). The sand is like powder, and beautiful 
blue sea, with great waves for the kids to go boating and body boarding in. And 
the ice cream and pasties are the best in the universe! But there's nothing 
quite like sitting outside the bar on the beach in the evening watching the sun 
go down whilst listening to a live band over a plate of nachos. It's one of  
those 'life is great' feelings. 

This Holiday has been wonderful, but it's also been a time of realisation. 
Realisation that when you have a disabled child- some places are just off 
limits!! We've alwAys wanted to go to St Ives- but a drive through and just 
looking to park told us it wasn't gonna happen. The same day we tried Hayle, and 
just the nature of the coastline being so steep with rocky un-wheelchair 
friendly meant that this, along with Perran sands beaches were also off limits. 
But I guess that's just all part and parcel of it. We'd come this way again. We 
had a day at Newquay which was lovely, and Perranporth is lovely enough to just 
go there. Or perhaps we'll do some research next time.

Hope doesn't seem
To like holidays as much as we all do. She liked feeling her feet in the sand, 
though not the sea. She did some good sitting on the beach too.  She certainly 
enjoyed her daily 'ice cream o clock'. And she definitely seemed to like 
sleeping in the bed with me and chris all week (there wasnt room for a cot in 
our room, and im not ready to not have her right next to me all night). 
But she's not that good at just relaxing. She seemed to miss the constant 
attention and stimulation. I even found myself doing her Brainwave exercises on 
the beach just to try to wear her out. 

A word that was 'emerging' before we came away, is now well and truly in Hope's 
repertoire. 'OUt'. As in 'OUt' of my wheels, 'OUt' of my chair. 'OUt' of my 
carseat. She's been very demanding!!! 
Fab though. It's such a big thing that she can tell us that she's had enough. A 
little tiring two hours into the journey home :0) 

Dear Tesco. Is it really too much to ask...

Dear Tesco

Is it really too much to ask...
For a trolley??? Like most of your regular customers, I spend between £600-£800 per month on groceries in your shop. Yet because I have a disabled child shopping is far from easy. Admittedly, shopping with a 2 and a bit year old, is never an easy task. But when your child is unable to race around the aisles, or is unable to physically sit in the trolleys, things become even harder. Until a few months ago, I'd put my nearly 2 year old daughter in the reclining baby seat type of trollies. But obviously these have weight limits, and my daughter reached this limit. Her legs were dangling over the edge of the trolley.

So now, my option is to push my daughter in her wheelchair around the shop with one hand, whilst struggling with a heavy basket with another hand. This means several trips a week, which I have to fit in around the various therapy and hospital appointments. This also means that on the weekend, after a week at work, my husband has to brave the aisles.

In November, I approached customer services with my dilemma. Knowing that a 'special' trolley would benefit not just me, but lots of families and their children in swindon. Back then, it seemed simple. A trolley would be ordered and arrive within 6 weeks. 6 weeks came and went, but taking into consideration December, I waited until February before asking again. At this point I met with a duty manager 'B' who said she had worked in other stores where there were trolleys, so she'd try and get one from there, plus order a new special trolley from head office. She took my number and said she'd be in touch. February, march ... April... Nothing!
So back to the store I go. I ask to speak to 'B', who is unavailable, so a male duty manager comes down. I explain the situation. He radios to 'B', then feeds me what I now know to be the biggest cock and bull story ever!! Apparently, there are no spare special trolleys ANYWHERE. Infact, they aren't even available to order anymore. They are in the process of designing one!! BS!!!

Feeling frustrated, I contact head office myself. Something I should have probably done at the start. On the same day, I had a call from the store manager 'S'.

He apologised for everything that had happened previously, and assured me that he had now ordered a special trolley. That it would arrive in store within 21 days, but in the mean time, he'd managed to get hold of one on loan from another store. Though he wasn't sure how he was going to get it to Swindon yet. But he'd of course let me know when it was in store. So I left it a week, nothing.. So I popped In and asked to see 'S'. He was available but 'B' was. Knowing she'd fobbed me off previously, she fed me more BS about how she'd not forgotten, was onto it, etc. And no, the loan trolley still wasn't in, but she'd let me know when it was. Still nothing!

With Hope being unwell the past few weeks, my focus upon this had slipped. But when I was leaving Tesco on thursday, I saw it. And what I saw was completely unsuitable! I know it's the one, as it's as described I suppose. But really it's so completely useless to us. It's really much more suited to a large perhaps autistic spectrum child. Or a large child who at least has control of it's head and trunk. It was basically a blue plastic almost school-chair like. Ok, it'll probably suit someone. But we are still no closer, to what is beginning to seem like the impossible.

Ok, yes Tesco, I could do my shopping online. Thanks for the other offer, to have someone assist me round the shop. But you know what? We are excluded from 'normal' enough. I'd just like to be able to do my shopping without it being a big deal.

Yours sincerely
Caroline

No more doctors now please!

It was getting pretty insane last night waiting for her too pee; we were trying to get her to drink all day, but with little success. We resorted to jellies. We were obsessively checking her nappy. we tried; sitting up, laying down, going for fresh air in her pram, then it got desperate, Chris had her sat next to a running tap, I was trying to massage her tummy and put pressure of her bladder, one nurse suggested blowing on her down below- then me and a nurse and Chris were all blowing!! Hilarious and crazy!
It got so we weren't even checking, we just had her nappy off, with the catchy thing attached to Hope, and we were watching, pleading for her to pee. One older nurse came and suggested splashing her with water, then said she'd try a joke to make her laugh first. I can't even remember what she said to Hope, but not long after she started talking, the bag was filling with yellow! There were claps and cheers all round! We finally left about 6:30.

We were told to expect Hope to be drousy for the next 24 hours or so, and that she may have a headache, but to watch out for signs of infection which can be caused by the lumbar puncture. She seemed pretty well last night when we left, and though she was a bit uncomfortable in the evening, I wasn't overly concerned. However, this morning she was hot. Hope doesn't do hot, her temp yesterday at the JR was 35.3 which Is perfect for her. I got her into the docs who checked her all over, and has given her antibiotics for a chest infection. I'm relieved she doesn't think it's related to the lumbar puncture, but still pretty sad that Hope now has something else. She needs a break! And so do I! I would really now please like to have a couple of weeks off of doctors and hospitals!

Oh and thanks everyone for the messages and texts. Sorry I can't reply to texts at the moment. In my lent promise to give up Facebook, my phone has taken rather a hammering!! I used 600 in just over 2 weeks- oops! Lol. But I get my new text allowance next week, plus... Not long till Easter... I'll be back on Facebook!!!!! :0)

Xxx

Please pee plonkey!!!

Everything's gone as well
As it could go. We've been well looked after, and Hope was even supplied with some fibre optic lights to play with. We had a slight hiccup in the beginning as Hope started coughing and sneezing just as the doctor came to see her. Then the doctor was then considering postponing the tests until she was better. But as I said to her, since November-ish I think, Hope seems to have come down with various viruses and infections one on top of another, so getting another appointment that coincided with a 100% well Honkey, may be tricky! After a thorough going over of Hope, she said she was reluctant to use the planned iv sedative that would have kept her asleep, but also carried a greater risk of respiratory depression, and would only use an oral sedative that wouldn't completely knock her out, but just make her a bit drousy. I did go
With her for her last lumbar puncture, but kind of assumed I'd not be able to here. The nurse asked if we wanted to go in with her, or offered the services of a play worker to comfort hope. As awful as they are to witness, I felt that the least we could do was to be there to comfort her.

Though she'd woken by the time of the procedure, she was still drousy, she gave a little fight as they tried to get her into position, and cried a bit as they took the spinal fluid, it was far less traumatic than her previous lumbar puncture. After that, 2 doctors and 2 nurses spent over an hour trying to take blood. Hope was fairly patient with them all pricking her trying to get the good veins. They took FOURTEEN vials of blood!

After that we went back to the ward. Hope had her vigabatrin in some custard (5 hours late due to fasting for the sedation). They were pleased with her recovery and that she was again alert and had eaten and not been sick. So as soon as she pees, we are free to go. They'd put something in her nappy when we first arrived to catch a sample of urine. It's still dry! We've been here since ten. We have been sat just waiting for her to pee for a couple of hours now. We keep checking and hoping...
C'mon Honkey.... Please just PEE!!!!!

On our way to the hospital of doom and gloom

We are on our way to the John Radcliffe. The hospital of doom and gloom; the hospital that told us that Sam had 50% chance of surviving his CDH, then 30%, the hospital that told us that Hope had extra fluid in her ventricles, then that she had a small cerebellum, that she'd most likely never walk or talk, that if this was his baby, he'd........... Without question.

I already have butterflies. Chris decided that this morning was the best time to first mention that he thought these tests were pointless, and aren't going to change anything anyway. this is a battle I've been having in my head for nearly a year now- since we last pulled out. Part of me wants to pull out again, but it's too late... And it does need to be done. I hope in a way, that we don't get any answers... but hope that we can rule out some of the more scary mitochodrial disorders. I hope with all my heart and soul and every cell we don't get a diagnosis that carries a life sentence with it. I don't want to know!!!! I guess I'm also hoping that we may find out some positives, that it's not all doom and gloom, that there may be something that they can try, a new medication, the ketogenic diet perhaps, miracle cure would be nice!!!

We won't find out today. It'll probably be weeks, or months. Most likely, today will be the first of many visits, perhaps trips to Great Ormond Street will follow.

Today she will be sedated for the first time. In a way I'm glad she will be, because the last time she had a lumbar puncture, she was awake, and it was such a traumatic experience for us both. I'm hoping they are just planning to use a short acting sedation like midazolam, and not general anaesthetic , but the fact we've had to fast her since midnight suggests the latter- though hopefully I'm wrong.

Poor lil thing, I've not even been able to give her her epilepsy medicines. I just hope we can get them into her early enough for it not to have negative consequences later on. Ironically, today, she does seem to be pretty much better. Though I suspect later, she won't be such a happy honkey.



Lots of love, luck and prayers needed please!

I'll update again later

Xxx

On the mend??

Today we had to go back in for a review and to have Hope's blood gases checked. It was hoped that her lactate would have come down again, then we'd all sigh a breath of relief that she was on the mend. Unfortunately it was still 3.8. Therefore the SHO (senior house officer) doctor couldn't discharge her. She had to consult with the registrar, who in turn passed the decision making onto
The consultant. All this meaning what could have been a quick In and out visit, was getting longer, and was looking scarily like we werent going to escape. After examining Hope, she agreed we could come home. Though she's still very sleepy, we've had a couple of moments of awakeness and even some smiles. She's not eating or drinking hardly. When we went in this morning, she'd had her morning medicine yogurt (that's just what we give her her cocktail of medicines in), that was hard work to get down her, but is not optional! Then a couple of chocolate buttons whilst Dolly was doing her ballet exam.

We came home late afternoon; hoping she'd now be hungry, I made her an egg Mayo sarni, which she gagged on and spat out, so figured I'd temp her with a milkybar dessert. She wouldn't even eat that!! We even fought to get calpol into her, nevermind anything to drink which is a struggle at the best of times.
Within an hour of being home, even Chris said he thought she'd be better off in the hospital, and I was thinking that he may be right. Hope then slept and slept and slept. I made up a big batch of her favourite butternut squash and pear soup, in the hope that we can try to get some fluids plus something nutritious into her over the coming poorly days. I went out for a run (I've missed that this last week) and was so relieved to come home to a Daddy who was very pleased that he'd managed to get her to eat some.
I was seriously considering (and I haven't quite ruled out) taking her back in tomorrow for iv fluids, but hopefully this is a step in the right direction.

On a less positive note... Her Nappies have been dry all day and night ( it's now 11pm) except for one wet one at 11am, so fluids may still be needed. Her mito disorder means that dehydration is a big no no, and the lactate being high makes this even more crucial.

So just hoping really she keeps taking these teensy steps in the right direction, and that we manage to avoid the children's unit for the rest of the weekend.

The consultant did say to bring her back if she's consuming less than 25% of her normal diet. I think we are just on the border now thanks to the soup. For what reason I do not know, since they can't seem to lower it, she said she'd like to repeat the blood gases next week. But I explained that we are due at the John Radcliffe next week anyway for all sorts of tests, which will include bloods of all sorts... And a lumbar puncture :"( trying very hard to put that to the back of my mind for now

Thanks for all the get well messages. I've passed them all on to her honkness

Xxx

That was scary!! 999!!!

Hope was unusually sleepy on Monday; a sure sign she's about to come down with something. We went to bed about 11 (hope was in with us). She started fitting. Not an unusual occurrence for , but when i noticed it was getting stronger rather than coming to an end, I glanced at the clock. 11:33. This is something that's become fairly instinctive now, knowing that there's a limit to the time she should be left fitting. Approaching the ten minute mark, I told Chris I was thinking of calling for an ambulance. Hope was still on her back, and then started choking. I lifted her up and she was really sick. SHE HAS NEVER BEEN SICK BEFORE. Then she was choking again though the seizure seemed to be lessening. We called for an ambulance. Hope's seizure seemed to have ended, but she hadn't recovered to her normal self. She was quite unresponsive and trembling and was having some jerky movements in her legs and feet ( leftover seizure activity). The first ambulance crew to arrive was a fast response unit in a car, so they called for an actual ambulance to come and take her to hospital . She seemed to quite enjoy the ride. Though I hated leaving Jordan in Max in bed , I kissed them before I left, and felt to sad knowing they'd wake up worried :"(

Hope was seen by a nurse upon arrival at the hospital bay. She started fitting again, and they moved her to a resuscitation room! A bit extreme I thought! And scary! But they said that's they'd bleeped the paediatric doctors and that's where they'd would want to see her cause they'd have everything they needed to hand. Her fit had ended by the time they arrived, though her little toe and foot were twitching :0(

They took bloods and arranged for her to be taken up to the ward. Her lactate level was upto 4.8 which is so so high and made me very sad ( it should be under 1.6, but Hope's is always raised, though last time it was checked it was only 2.4). It's an indicator that something's definitely not right. Her temperature was 37.8 which is really high for her. Because of the mitochondrial disorder her normal body temp is only 35, and has never been above 36.

She had another nasty fit on the ward, and the doctor was just ready to give her the rectal diazepam to knock her out when the seizure lessened.

Still not knowing what's really wrong,they decided to treat her with intravenous antibiotics, and iv fluids to treat the acidosis in her blood.

By the time this was set up, it was 4am. The recliner chairs to sleep in are far from comfy, and before I knew it, it was morning.

She was on hourly obs, and continuing her iv fluids until the doctor came around 1. He didn't really know what's wrong either, but agreed that we could go home in the afternoon providing she kept her canula in, and her lactate had come down and her hydration was ok. So more bloods. The saying 'like trying to get blood from a stone ' springs to mind. Poor lil honkey is like a pin cushion. Later in the afternoon we were told that the blood gases machine wasn't working, so we could go home for the night and come back in first thing in the morning for more iv fluids, antibiotics and to repeat the blood tests.

Hope slept all evening, through the night, and was still sleeping when we went back in at 9. She had her iv's, then was seen by the junior doctor. She wasn't too happy with her or that her lactate was still 3.8, and I was concerned that she was so sleepy and still jerky, so we had to wait for the registrar to see her. The registrar felt that the lactate, sleepiness and jerkiness were all related to her having some kind of infection, and hopefully she'll improve soon. We were discharged, but have to go back on Friday , by which time , hopefully, she'll have improved, and hopefully her lactate will have come down. If not, then I think she'll be admitted again.

She's Still fast asleep (6pm). I miss seeing her smile. I've tried putting her in the swing, and rowing the boat, and blowing her with the hair dryer- all her favourite things, but no smiles. Though she did manage some ice-cream from the ice cream van after school.

Botox!

Botox

There's been rising concern again over Hope's tendons in her feet and ankles. Despite wearing splints (which are an ongoing nightmare in themselves) as soon as she's out of them (which she often manages to escape from them herself) her feet point outwards again. This makes standing (supported obviously) uncomfortable and well... They just aren't how feet should be. The more she does it, the less the range of movement becomes.

We are just going through the process of having new splints made. It was at our last appointment that the dreaded casting was mentioned again. Last year she spent 5 weeks in plaster casts from her toes to the tops of her thighs. This process stretches her tendons. But it's a nightmare. She cant do her favourite actives of rolling, swimming and soaking in the bath. Clothing is limited. But mainly, She's so awkward and heavy with them on. She was last year, and she's almost doubled in size since then.

It was left that we'll see how she goes in a few weeks, and see how she gets on with her new splints when they arrive next week.

On Tuesday we had our regular 3 month appointment with Hope's main consultant (and wonderful man) R. He gave her a thorough check up, and was generally satisfied with her epilepsy, her spine and overall health. Though he remarked that the bout of coughs/colds/ infections she's had recently may be linked to me stopping breastfeeding, in November of all months!

He's never really shown much interest or concern over Hope's feet and ankles. Leaving that to be dealt with by the physios and appropriate doctors. However on this day, he was very concerned over the tightness of her tendons and the inflexibility of her feet and ankles. He pushed twisted and pulled my poor baba around till she screamed and tears ran down her face :"(
But, for the first time ever, Botox was suggested. I've heard of it being used for children with cerebral palsy quite often, but had never considered it for Hope. But R Hope's that by injecting botox into the surrounding muscles, they might relax somewhat and relieve her of the tightness. So, she's now on the waiting list- which is 3-4 months or so. So hopefully in the mean time, we can keep the plasterers at bay!!

New wheels! And more nice news

Far too much time has passed for me to try and catch up, so I guess it'll be sporadic postings every now and then. And that's probably /hopefully a good thing, because in hindsight, this Blog is often my therapy /outlet at our darkest patches. And we don't like those thank u.

Well Hope hasn't exactly been a picture of health recently: the flu jab in december really took it out of her, and since then she's had a chest and throat infection. Though she's on the mend, her illnesses seems to be a vicious circle of being ill, not eating, therefore not taking her epilepsy meds properly, getting dehydrated, and therefore having more seizures and being more lethargic... Not eating etc.

Anyway, this is supposed to be a happy post. Her wheels are here!! A momentous occasion!
I was worried that they'd arrive, and that even after all the effort that went into getting them, that perhaps I wouldn't like them as much as I thought I was going to. Or that I'd have some issues/ personal
Battles to wrestle with over what it actually is/ represents. As in, it's not a pushchair. It's a 'postural seating system' or wheelchair. It represents That my honk can't walk. That she's different. However, it is so splendid, I am pleased to say, I have no such issues. It's nicer than I remembered, and seeing Hope so comfortable in it, just makes it even better :0)
Thank you doesn't seem to cover it really, but thank you so much to everyone that made this possible!

What's more, due to the late (but rightly so) contribution to the wheels by wheelchair services (they finally agreed to take back the useless wheelchair they supplied, and give us it's value towards the stingray), Hope now has a nice amount of money left in her bank account. I decided to get some more extras for the stingray; an extra harness, a grab rail and a play tray (expensive extras totalling just shy of £200). With the remainder of her money, I intend to buy her some 'switches' and switch toys, and a special swing for the garden. Hope LOVES swinging, but her head control isn't strong enough to cope in ordinary swings.

More nice news: I've mentioned the rainbow trust before. We have S from there help us when we need it (though I'm pleased to say we hardly ever do these days- though I know she's there should we need her). Anyway, last year, I shared Hope's (and our whole family) story, and agreed to have pictures taken for the rainbow trust to use in their Xmas appeal. Seeing it all in print, I have to admit, I was a bit gutted, just cause my family life was made out to be a real sob story , and I really don't like people to feel sorry for us, when actually despite everything these last few years have thrown at us, I do feel blessed ( most of the time anyway). Today I received a thank you note in the post from the rainbow trust, thanking us for allowing them to use our story, and saying that our story had already bought in more than £17,000, and they are expecting more! It's so nice to think we've been able to give something back to a charity that's helped us so much in our times of need.

Anyway, a mahoosive thank you from me and Hope to everyone who sponsored us and helped to
Pay for the very best wheels that money could buy!

Xxx