Brave Girl.

Well PM has finally had her fundoplication/G tube operation. She was in hospital for exactly 7 days and coped so well with it! She was amazing. Even when placed into isolation as she had diarrhea, she just seem to bounce back so well after such a big op. I'm so proud of her!
There was no complications and the operation took about 3 and a half hours. Very pleased with how it went and she had the same surgeon that did E's which was even better. Cant say I didn't cry when they put her to sleep using the gas because I did! She fought so hard bless her and even when half asleep was still fighting against it. She's been home since Monday and is loving baby food, even E seems to have finally developed an appetite after another few mths of not eating anything.
E is currently on the waiting list to see an orthopaedic Dr as she has knock knees and at her next clinic appt we think she might get an x ray as her tummy looks quite swollen and they want to make sure everything is ok with it. They had clinic last mth and E had gained a whole kilo in 1 mth! Was very surprised as she hadn't been eating but very pleased and even PM had gained a bit of weight :D
E is still loving nursery, she loves it even more as grandad normally takes her and brings her home. I'm sure she loves grandad more than she loves mummy & daddy haha. We went to the beach a few weeks ago for the day, the girls had a fab time and got spoiled rotten. Hoping to go again next week before the Summer holidays kick in! Then we want to go to the sea life centre at some point, the girls would totally be fascinated with that. They both love animals.
PM had a fantastic 1st birthday and was spoiled rotten, her cake was so beautiful to, mmmmm. Cake :p Its almost been a year now since PM herself was diagnosed! She's now crawling, standing up with help and trying to say words. She's a clever little munchkin. E's speech is also coming along amazingly, she can now count to 20, copy many animal sounds and probably say about 50 words if not more. She went on the donkey ride at the beach and LOVED it, she didn't want to come off even after 2 go's. She must have gone on every kid ride possibly and even went in the sea with mummy. PM loved playing in the sand. It was a really nice day out, cant wait to go again :)
So think that's it really, no meds have been changed recently which is fab news but we have clinic next week so that might change yet but hopefully not. In 6-8 weeks PM will have her tube changed into a mic Key button like E's which is so much easier and better and wont get in the way when she's trying to crawl. Next week she has some stitches out and another blood test, she had one every day while in the hospital poor baby. Her hands are so bruised its horrible. The nurses and Dr's loved her, everyone kept saying how beautiful she was :D We knew most of them from when E was in as she was on the same ward so was quite nice to see them again and we gave them a card and 2 boxes of chocolates when leaving as a Thank You as they where fab. So lucky to get such nice renal Dr's and nurses.

What's Been Happening ...

Well finally have some good news. PM should get her OP in the next few weeks as the surgeon has put her on the priority list :) Only thing is her 1st birthday is in 2 weeks and have an awful feeling she might be in hospital for that! If she is the nurses will be enjoying her cake I ordered weeks ago. The Drs & Surgeon is doing the G tube and the fundoplication at the same time as there was a bit of a hoo haa about the fundo not being done by another Dr but her specialist put them straight and said she needed it. They are worried about her growth, said she's missed out on a vital part of it in her 1st year but they are hoping after the operation and she starts gaining weight she might start growing, if not much after 6 months they will consider GH (Growth Hormones). She is 1 soon, weighs 15lb 15oz and is still wearing 6-9mth clothes and some of them are slightly baggy! She is crawling loads now though, so proud of her and love the two front teeth she has. Her Cystagon has also been increased lately due to her level being higher than they like and her phosphate was low again so that has to be upped now to. She did really well at her last blood test and hardly cried, she's just awesome!!
E's doing good so far to, a few of her meds have increased as well like the Cystagon as her level was raised. They changed her micKey button recently as well as the one she had had gotten too big and was rocking too much and causing her some pain and overgranulation. The new one fits nicely at the moment and the Drs took a swab as it looked a bit red and sore and they wanted to make sure there wasnt an infection, plus she likes to touch it a lot. She is also being reffered to Orthapeadic Drs now as she walks funny and might need special shoes at some point. Her weight has dropped slightly since the last clinic, she currently weighs 23lb 10oz. She also has finally been given a Nursery place for a few hours a day every weekday if we wanted, we're just on visits at the moment so she gets use to it and settles in. She loves Stay & Play but we are always there with her so she isnt use to being left alone. I know I will cry when I leave her for the first few times!! She's also doing fab with her speech, taught her loads of new words. Unfortunately her eating habits have subsided. She tends to eat nothing all day so im really thankful she has her milk feed at night, same as PM. E did good on her blood test to, cried but unfortunately she knows whats coming now whereas PM isnt too aware. She got to go out with Grandad after anyway to the park while PM came shopping with mummy & daddy so everyone was eventually happy again!
So its all go go go again, hopefully my next update will be celebrating the fact PM has had her op as is thriving! Fingers crossed.

A Year Ago This Month.

Just come to update my blog. Thought it was time I made a post!
Girls are both currently well, we where at clinic a week ago and they had both gained a little weight and grown a little bit to which made mummy happy. P-M's appointment has come through so she'll be seeing the surgeon soon about her G tube/Fundo operation and hopefully a few weeks after that we'll get the date for the actual operation. Just hope its not too close to her 1st birthday which is in May.
Cant believe its been almost a year. Its also been a year this month that we got E's diagnoses and our lives dramatically changed. The nasal tube went in, the meds started and the fortnightly clinic appointments began. I was 7 & a half months pregnant with PM, seriously dont know where the time has gone. Has zoomed by!
So much has happened since then. We had a baby, E got her G tube/fundo done, PM was diagnosed at 7ish weeks old and then had a nasal tube put in, moved house, didn't have adequate heating and appeared in the local paper twice! They've both been in & out of hospital with viral infections to.
PM is still sick, 2-3 times a day so loads of washing to do every day to. E's been going to stay & play and been having a great time there, PM's now trying to crawl :) She's still quite small and in 6-9mth clothes. Ive been put on anti depressants last month and they are helping, mood swings are alot calmer and I don't cry so much now.
The other half & I have recently decided to organise a Fun Day for Cystinosis Foundation in the next few months so hopefully organising that wont be too stressful! Have PM's birthday party/stuff to get all sorted to but think we're almost done with that.
We sure have had a roller coaster of a year thats for sure but if I could turn back time, I wouldn't change a thing. It's hard at times with meds every 6hrs, eye drops every few hrs, diarrhea and super explosive nappies plus all the washing but I love my girls so much. They are an inspiration to me, never grumble or moan; even with the blood tests every 4 weeks! They are super stars and amazing.

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.