Friday, 6 April 2012

Credit where it's due : the ketogenic diet

(I'm well overdue in posting this and actually wrote this on 14th march)



Though I hoped that Hope would be one of the 'miracle responders' to the diet, I was reluctant to start singing it's praises too soon. Wouldn't want to look a fool :0) but now there's no denying it (not that I'm a fool) its making a BIG difference!
So much so that I've had to start writing down all her new achievements. There are that many!

After 3 days Hope began reaching for, batting at (a few days later ) grabbing the mobile that had been hanging above her cot for 3 years!!!

Within a week, she seemed to be lifting her arms to be picked up. Now there's no denying it. You can say 'c'mon then' and she'll lift her arms up.

All of a sudden she has hand-eye coordination. You can wave something in front of her, she'll look at it, and if she wants it, reach up and take it.

She has a real increased awareness for cause -effect. She used to hit things, but not as ferociously or as purposefully as now.

Hope has never been a good drinker. We had to remove valves in 'non-spill' cups to make them free flowing (so we'd drip/pour fluid into her mouth, but it needed to be thickened otherwise she'd cough and splutter on it. But when i recently bought her a replacement cup, i thought ...'maybe, just maybe'.. And left the valve in. She did it!
I also bought a 'nuby' cup. If youve ever tried drinking from one of these, you'll know you need a good strong suck. And she can! And what's more, she doesn't even need her drinks thickening when using this cup!( This is probably the biggest one for me).

Many of you will know that Hope used to be able to sit (albeit briefly and she would eventually wobble and topple over)but that when she had pneumonia, then several more poorly spells, she completely lost this skill. Well, it's emerging again. She's only doing about 20 seconds or so, but I believe she will crack it again.

And her most recent development, is Putting things into her mouth.

To the parent of a typically developmental child, (I've been one myself) these are not achievements , but 'givens'. ..things that just will happen. But when you have a child like Hope, you celebrate every achievement. Though usually one doesn't get to celebrate so often as we are at the moment. Usually, new developments, come slowly, and take a lot of work and practice.

What's even more amazing, is that during the past 6 weeks, Hope has been poorly in hospital twice (one for the peg op, and then we had a 3 day stat just last week due to fever and seizure activity). Usually during periods of illness, Hope regresses. Yet she's not regressed at all!

Oh and just one more thing, when she was in hospital last week, we checked her lactate (normally 3ish, can be 5-6 if poorly).. It was 1!!!
The best it's ever been. And within the normal range!!!

Thursday, 16 February 2012

Peg (and more than we'd bargained for)

Pre op we met with the surgeon, who asked whether we'd been told Hope needed a peg (feeding tube) or whether it was us that wanted one. I pointed out that it was hardly every parent's dream :0/

But this is why Hope had the op:

1-When she's poorly, she shuts down and refuses to eat or drink, which lands her in bigger trouble. And ultimately hospital.

2- at the best of times, Hope does not drink enough. At best 400-500 ml a day. Less than half of the recommended fluids for her weight /age (100ml per kg body weight. So 1.2l)

3- aspiration. It happened. She got pneumonia. We had a study done that shows She's 'at risk', 'showing signs of early aspiration'. Personally, I think she's ok most of the time. But when she's poorly, or refusing fluids.. It's more likely.

4- I'd rather now, a planned surgery, than wait til we really need one.

5- though hope will eat orally, it's a very useful back up tool for delivering the ketogenic diet.

6- it'll stop all of her foods tasting like poison. Because previously the only way we could get them into her was in food to try to disguise. But I know she tastes them.

All that said... There was one major concern. The general anaesthetic. Last time (just the skin and muscle biopsy) there were several complications following, including the pneumonia , acidosis, dehydration etc.

However, now we had the diagnosis, so much more was in place. A plan from the metabolic specialist at the evelina, which included pre and post op fluids, no glucose. Plus she was in the ketogenic diet...

What's the saying? 'the best laid plans...'

These are the things we didn't expect:

I didn't expect (though should have) that we'd have such a nightmare getting Hope canulated. It's never an easy task, which is why I gave the junior doctor sent to do the task one chance. Obviously he failed. 2 senior house officers tried and after several bust veins and a bruised baby girl from so much squeezing, they admitted defeat. Round 3- the registrar. Determined as she was, I had to insist she stop after about 6 attempts. I think she was relieved, though perhaps a little defeated. Eventually a surgeon was successful after a couple of attempts. Not a nice experience for mummy or honkey though. And it was mentioned by a couple of doctors that perhaps we should consider a 'port' (a permanent line for IV access).

Neither We didn't expect Hope to come back after the general anesthetic and not be able to rest because of seizure activity jerking her awake. 12 hours later she was given via IV Phenytonin to stop the fits.

We didn't expect the Phenytonin to cause her limbs to tremble.

When she was still fitting the next day, still unable to rest and recover post op, that they'd give her midazolam to sedate her and control the seizures. Then.. She started de-satting and needing oxygen.

We didn't expect that she'd be sick anything that went into her tummy. Even 10mls of medicines were coming back up. This went on for days and she wasn't able to tolerate any feeds via her tube. Eventually chris bought up bags of her keto groceries and we took over the hospital fridge. Hope began tolerating small oral feeds eventually.

What I really didn't expect, is that after being discharged later than planned, that nearly 3 weeks later we'd still be struggling with sickness and reflux. Problems we didn't have pre-op.

I really didn't expect, that nearly 3 weeks later, I couldn't put more than 50mls in the PEG without her throwing up :0/

I didn't expect to question whether we'd done the right thing. This was meant to be a solution. A prevention.

I'm Told these are teething problems. I really hope so.




Monday, 30 January 2012

Day 1 keto

I'm reassured it'll get easier. Good job. I feel like I've spent the whole day examining nutritional values, weighing, cooking, feeding, then starting over.

This was not helped when I left 21g of de-crumbed fish fingers to cool down, to later discover the cat had stolen them . So I had to do them again.

Hope has been a little trooper. Obviously! And taken it all in her stride. No issues *touch wood*. Hasnt even cried when I've pricked her 3 times to check her glucose and ketones.

No ketones as yet. I must be patient. It'll take a few days for her metabolism to switch energy source. Though I'm sceptical as to whether the 2:1 ratio will be enough. We'll see. I'll be keen to move up to 3/4:1, but the dietician feels this is the safest way to do it as an outpatient.

Sunday, 29 January 2012

Some lasts....

Today's been filled with mixed emotions as I've given Hope her last favourite foods. Her last roast dinner (as we know it), followed by chocolate brownie dessert with ice cream. Then this evening, a petit filous 'little dessert', and some finger of fudge. I'll miss her little concentrating on eating chocolate face. Hopefully it'll be worth it.

Saturday, 28 January 2012

Happy 3rd birthday Honkey baby

Happy birthday to my amazing, brave, inspirational, beautiful little girl!

I must admit, it's a relief to get to 3. There were times I've wondered ...

Many people have said in the approaching weeks 'wow, doesn't time fly'/ can't believe she's 3' etc. However, it feels very different from here. The tiny baby born 3 years ago, seems like a lifetime ago! So much has happened!

This is how our day rolled: as happened at Xmas, Hope became quite overwhelmed by the early morning present unwrapping. Jordan and max were great, taking it in turns to help her open her gifts, then making a big fuss of the gifts for her. They are amazing kids!

Since Hope got pretty much everything she needed for Xmas, there wasn't much left to get for her birthday. We got her a cd and tape player (yes we still have tapes! Hope can control them using her 'switches' whereas she can't with CDs ). A cd AND tape player, that was pink or purple was impossible to find, so we bought a nice shiny black one and decorated it with elmo stickers. We also got her some new books (she LOVES stories), and a leapfrog 'my pal violet' interactive puppy that we've programmed with her name, her favourite food (icecream) , her favourite colour (yellow) and favourite animal (giraffe) and favourite songs 'twinkle little star', 'if you're happy and you know it', 'wheels on the bus' and several more. It's a pretty cool toy! Though Hope rated pretty highly among her gifts.... A piece of ribbon (that was used to wrap). She properly played with it for ages.

Hope will be looking fabulous this spring/summer since we asked family and friends to buy clothes as she really didn't need anymore toys. I'm
Looking forward to her wearing all her new outfits. I find choosing Hope's clothes each day much more exciting than choosing my own.

Hope wouldn't appreciate a big party, so we pretty much spent the day indulging her in her favourite things; stories, songs, rolling on the floor, cuddles, listening to music, and a nice long-very -splashy-get the bathroom floor absolutely soaked- bath with mummy.

Of course we sang happy birthday and helped Hope blow out her candles on her cake. I was wishing very hard!!!


I feel blessed to have had Hope for 3 years, but I want so many more! It's not an easy, carefree life, but it's our life, and she is the centre of our universe. She's one tough cookie, who never ceases to amaze me how she bounces back, how she beams that amazing smile, how she is always learning and developing (in spite of the diagnosis). A lot of people could learn a lot from her! I know people do. I know she's taught me more than I'll ever be able to teach her. She's my little honkey hero.

She's doing a fine job of living up to her name! I just need to remind myself that sometimes.

Love u so much my honkey baby <3 xxxxxxxxxxxxxxxxxxxxxxxxxxxxxxx

Thursday, 8 December 2011

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). )