Still Going Well!

Girls had clinic again recently. E has gained yet another kilo in just 6 weeks, so proud of her. Dr/Dietitian suggested cutting down on her overnight feeds now to see if she gets more interest in food, doubt it will happen but we shall see. She was on 800mls overnight but slowly cutting it down, now on 750mls and soon will change it to 700mls. All her levels where pretty good, KCL has gone up an extra ml but her Cystine level was excellent, below 1 which is the lowest it has ever been! She still has quite a bloated stomach but they've investigated it enough and just think its full of air. She is 3 on Oct 22nd :D Cant believe how fast the time has gone.
PM is also doing good, her Cystine level was also below 1 which is fantastic. She's started on a new medicine called Carnitor, she isnt really growing much so they are seeing if that can help and she's been referred to Growth Clinic, she might be starting Growth Hormones a lot sooner than we all expected. She is currently 8kg and not much change in her height.
Both girls had eye clinic too a few weeks ago, E's crystals haven't gotten worse but they ha vent really gotten much better where as PM's have cleared up a little which is also excellent news! Back in 6mths for eye review and got a 6 week break again from clinic which is always a good sign.
We where all due Flu Jabs last week but we've all been full of cold so they had to be cancelled, hopefully get booked in next week for them. Last thing I want is girls in hospital again, last year they had a viral infection which put both of them into hospital at the same time. They got to share a room together (quarantine) for about a week haha. Drs recommend they have it and I got flu last year when they where poorly and don't want it again, it was awful!
We all had a fab time in Wales for our holiday, girls really enjoyed going to new places everyday and as usual E loved the beach.
E's speech is getting loads better, she can count to 20 now and loves to sing. She's a right little Drama Queen. No words from PM yet but she's trying to walk and she loves eating. She's on her new milk now and tolerated the change really well, she gets almost 700mls a night.
We have another appt in November for E to see the surgeon that did her Fundo/G tube op, think he's interested in her bloated belly and wants a look. Well that's about it for now!
Just glad to post some Good News.

Going Well!

Not to jinx anything but the girls where at clinic recently and the results where fab! E has gained another kilo in 4 weeks and now weighs 26lb 10oz, she's also grown a few cms. PM's results weren't the best, weighs 17lb 9oz so hasn't gained much and has apparently shrunk. Bloods where good, PM's KCL needed reducing by 1ml so thats good an no chances for E except being started on the Domperidone again for her bloating.
PM has done fab with her G tube, it recently got changed into a mic Key button and is doing good. It was really badly over granulated at 1st but the Community Nurses have been coming by every few days and using Silver Nitrate on it, its cleared up so much and looks good now. When changing her over to the button PM was fast asleep and only woke once to see what was going on! I was very impressed she slept through it all, she's one brave sweetheart that's for sure.
We're off on our 1st ever family holiday next month, I'm dreading it in some ways as I'm scared I'll forget meds or their machines or milk. Don't fancy getting all the way there and realising, that wouldn't be good haha.
PM is crawling and standing up unaided, also walks if you hold her hands. She got her 1st pair of shoes a few weeks ago and they are super cute, know she had grown a little because she finally could wear shoes!
E's growth spurt is fab news though, this time last year she had just reached 20lb so gaining 6lb in 12 mths is pretty good for her, I think anyway. PM loves her food unlike her sister, she eats baby food and finger foods so I'm quite surprised she hasn't gained more weight but next month they are changing her onto a milk with more calories in to see if that helps. Dr said if not much improvement in 12 mths with her growth they are going to consider Growth Hormones.
Next appt isn't for 6 weeks now which is also a plus and a good sign. Keep gaining weight and growing my gorgeous girls, mummy is super proud of you both :)

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