The Things We Do For Love.

Well I posted yesterday about shaving my head for Cystinosis Foundation and raising money. Reached the £200 last night so earlier this afternoon my partner cut & shaved all my hair off! We have raised £265 so far and have said if we reach £300 he will let me wax his chest for the very 1st time and if we get to £400 his legs as well. So will be excellent if people keep donating! I really want to cause him some pain haahaa.
E wasn't too sure at 1st but after it was done she came over for a cuddle and slapped my newly shaved head a few times. PM doesn't mind it as long as Im not wearing my glasses at the same time, she doesn't like me in them haha.
Pictures being added of before & after :)

Charity Event.

I have decided to raise some money for Cystinosis Foundation UK by shaving ALL of my hair off if I raise £200+ on my Just Giving page. ALL the money goes directly to the charity using the link I will post.
My hair is currently shoulder length as some of you might know. If it was longer I would have raised the target amount haha. Please, even if you can only spare a few quid it would be greatly appreciated. Your donation could help find the cure my girls greatly need.
Thank you! x-x

http://www.justgiving.com/Jessica-Kemp?ref=nf

Just a little update really. Girls are both good at the moment. Clinic appt went really well, E is now just over 22lb and PM is almost 15lb, both have a grow a little to which is great. E still on 9 meds & PM on 6. There has been talk of Growth Hormones for E when her height starts to level off & PM needs a PH test before she can have her G tube operation/fundo.
Both girls have recently gotten over Viral Infection which really knocked them back, PM a lot more and she took ages to get back to 100%. They both ended up in hospital at the same time so they kindly but them in a double isolation room together which was loads better for us to. PM was sick 16 times in the space of 4-5 hrs hence why she ended up in hospital on IV fluids for a few days. E was the same but she cant be sick thankfully because of her Fundo. Was just nice to get them home eventually, hate the hospital most of the time. They have to watch over you do meds as well which always puts me off and makes me mess up!
PM has her eye examination beginning of March, I'm hoping the crystals still aren't forming in her eyes yet as it's mad enough getting eye drops into E eyes, let alone doing both girls every 1-2 hrs while they are awake! E's speech is coming on slowly to, she can say quite a few words now but still wont eat properly. We've been advised to stop feeding PM solids until after her op as she just vomits after everything except milk & water. I hate feeling that we are starving her but also cant have her being sick too much as this will lead to de-hydration very quickly for her & weight loss which I don't want to see as she's tiny enough at 8 mths old!
Can't win either way which seems to the normal in this household with the girls lol.
Just letting people know I've deleted my Twitter as was getting bored of it & didn't really use it much but I will blog every so often about the girls progress etc.
Just glad since the Viral Infection they have done well & improved some, always makes me happy knowing they are doing really well. Just hope it continues!

Been 2 months since my last update so thought I'd best get one done!
Girls aren't doing too bad, they have recently been quite poorly though with flu & E had UTI infection on top of that which made her worse, her temp reached 41.3 at one time. They both spent a few days in hospital, some of them at the same time! 1st time they have been together like that, I doubt it will be the last either. I made the mistake of taking E to A&E when I should have just gone straight to renal ward, I got there at 10am and they didn't get in touch with her specialist until gone 5pm so she didn't get on a ward until just after 8pm! Then the next day PM wouldn't stop being sick so she ended up staying in to, 16 times in the space of 4ish hours she vomited! They where eventually placed in an Isolation room together for a few days.
At their last renal appt both girls had grown & gained weight so was very pleased with that. E's Cystine level has alot higher than normal though so she now takes 2 capsules of Cystagon every 6hrs. She now on 10 meds to and PM on 5. PM is catching up far too fast for my liking! She's very dehydrated at the moment to and I think she's lost a lot of weight since being sick, poor baby.
Community Nurse is due this week to change E's G tube for a smaller one, so not looking forward to that. We missed PM's appt with surgeon for hers but Dr's said they will try & sort something out as we where in hospital with them both. She will probs also have the fundo like E has so she cant vomit.
Poor girls are all covered in bruises from all the blood tests they had to have while in hospital, E's foot very bruised as they put the cannula in there as they didn't have much luck with her hands :(
Finally had our central heating fitted now though so glad we're able to keep our Princesses warm. Their rooms where freezing, we use to have to put PM in bed with us in the early hours just to keep her warm.
There has been a new page started on FB to get everyone together who has Cystinosis or knows someone who does and its a fab little group! Cant believe there is over 200 members, glad to have more people who understand exactly what we deal with every single day. Its really nice and everyone so supportive & lovely. I'm sure when the girls are much older (and if FB still around lol)they will find it very useful and be able to get more support and make new friends etc.
Dr's recently mentioned Growth Hormones for E if her height starts to level off but so far she is still growing so very pleased with that. She even ate some spaghetti bolognaise the other week, off my plate! Was very shocked but it was lovely and bought a few tears to my eyes haahaa.
Christmas was very good, was nice to be with family and the girls go so spoiled we had to have another xmas on Boxing Day just to get all the presents opened lol. Both girls where very well then as well so made it even better.
The girls where on the front page of the Yorkshire Evening Post to on Jan 4th 2011 due to us not having any heating since moving house, was so shocked to see they had made it front page though. Then a follow up story was done last week and a few days later a lovely woman had wrote in about the story, was nice to know there are other people out there who care to.
And here's me rambling again! Main things are the girls are now getting better after their visit/stay to the hospital so we're really pleased with that. PM is taking longer but she is still only very small bless her.

Hello. Thought I'd give everyone an update as havent since August! Girls are doing well at the moment but I know this can change at any minute. E has gained some weight & finally has reached over 20lb!! I was so pleased, she hasn't been 20lbs since just after her 1st birthday so was a very massive milestone :) She now finally has her Mickey Button in to, that was done about 2 weeks ago now & it's going good. She doesn't have much of an appetite again and hardly eats anything but that also changes from time to time. She now is fully walking to, which I am so proud to see. It brings tears to my eyes, I know that probably sounds strange but with all the struggles she has been through with the rickets and what not it is truly amazing!
P hasn't really changed, she has gained some weight but really slowly and the Dr's aren't too happy with it. They have said there is a 99% she will also have the operation for the button like E did but not until she gets a bit bigger & gains more weight. She still has her nasal tube in, when she isn't pulling it out! She still gets dehydrated alot to and she is a very clingy baby lol.
P is on ranitidine & the Cystagon still and nothing else at the moment. E is on 8 meds still to, inc the eye drops. They will both be due there 6 monthly eye check ups soon, really hope P doesn't get the crystals early like E as she'll have to eye drops to!
Hopefully things will keep going well now, got all our fingers crossed :)

Sad News.

Feeling quite sad today as read some bad news. A man in the US died of Cystinosis recently, he was only 25. It was really heartbreaking to read as I started to think about my girls and how it would kill me so much if either of them died before me! I couldn't bare it and started to cry.
These girls are my everything!
I have good days & bad. Some days I don't let it bother me & just get on with it & others I can hardly function as my head is swarming with all sorts.
P's doing well with her Nasal tube though, seems to be feeding much better to since we changed the teats to a size 2. She's not due back to clinic for 3 weeks :) E went yesterday for her clinic appointment and they are really pleased as she is growing & gaining weight. She now weighs 20lb 1oz and is 76.2cm! Since her operation she has been eating more, she was 2 Weetabix in the morning and normally always eats it all. Then a stage 2 jar for lunch & dinner and sometimes a few yogurts or ice cream. I'm really proud of them both.
I am excited though that there could be a meeting soon (in 2011) in the UK for people to meet up with other people who have Cystinosis or their children do. It will be fab & I know it will benefit us loads!
We've been doing well recently with the meds, finally got ourselves into a better routine with the eye drops & Cystagon. Both girls having it every 6 hours now like they should and E's drops every 2 hours. E now has 5mls of Cystagon and we've to put it up an extra ml every week until she is on 10ml which is basically 1 tablet of Cystagon (50mg) every 6 hours. P is on 2mls, no plans to raise hers yet as she still a sicky baby, even on the Ranitidine. So thankful P doesn't need the eye drops yet to, doing 1 is bad enough!
E's been having her over-granulation sorted. Community Nurse came the other day and put some Silver Nitrate on it. It seems to have helped a bit but there's still a bit left so we're hoping the cream will keep helping rather than having to use the Silver Nitrate again. She's also been started on Sodium Bicarb, 5.6mls twice a day. Cant remember if I wrote it down last time I blogged.
Some days I feel so depressed but then I just think about what my 2 Princesses must be going through and then I realise I have no right to feel that way really, its them going through the constant change in medicines, the blood tests, the tubes, the sickness etc etc.
Luckily E hasn't been sick since her Fundo operation so that's a big improvement for her & us.
I know not many people follow this blog & I have no idea how many actually read it but if you could just spare a £1 (maybe more if you're feeling grateful & generous lol) and donate it to the Cystinosis Foundation (UK), Me & my daughters would be eternally grateful. They only have funding through donations and without donations they cannot fund research into new drugs and HOPEFULLY one day soon a cure ...
I don't want this horrible disease to claim anymore lives. I hate IT with a passion. Wish I could take it from my girls - they don't deserve all this. They haven't even had chance to experience life. No one deserves it in fact ... horrible, nasty thing :'(
R.I.P Preston Towriss & everyone else who has lost their life to Cystinosis. You are always in my thoughts as are your family ...

P's nasal tube.

Well P now has her nasal tube in to. She had to be in hospital for 3 days. She still doesn't drink much milk and we cant feed her too much by the tube as it makes her sick. She had her eye test on Tuesday and luckily there isn't any crystals forming yet so eye drops aren't needed, she has to go back in 6 months to be re-tested. The Drs thinking of starting her on Ranitidine soon as she vomits quite a lot.
E's also been started on another med recently, bringing the daily total to 8. Sodium Bicarb needed twice a day at 5.6mls. Her G tube is doing well except she has bad over-granulation on it so got some cream yesterday for that. If it hasn't helped in a few days they will probs freeze it off! The fundo has worked fab, she hasn't been sick since having it done so really pleased with that.
It's likely P will need a Gastrostomy to but it wont be until she is bigger & weighs maybe, maybe about 6-8 months if she puts on weight now and starts growing lol. She 12 weeks old :) Still in 0-3 months clothing bless her.
We might get some good news one day ... lol.
Before & after pics :)