Hello!

Well sorry I haven't posted in a while. Had no laptop but then remembered can use the app on iPhone lol.
Trying to remember everything that's happened lately. Mostly it's been good stuff really. Few colds for both girls but have manage to keep them out of hospital!
E had a kidney scan few weeks back, the quality was poor but her dr says her left kidney is damaged and only doing roughly 32% of the work and the right is doing the rest. Nothing can be done about it but its nothing to worry over either really. Dr says she could have been born like that or it's from the Cystinosis/ UTIs she gets. But she's fully potty trained during the day now so not using nappies should help loads in that area. She's also losing weight over the past few months which the Drs are now concerned over. Of she loses anymore they want her to be admitted. She recently had ear infection in both ears to but that cleared up pretty nicely and after some amoxicillin. Her eyes aren't really doing too well lately either, she's been wearing a patch everyday for a few weeks to strength one eye as its really weak and not good! She's back in 2 weeks to be checked again and see if its improved with a patch. Apart from all that she's doing well!
PM has gained some weight thankfully, not growing height wise so discussing the possibility of growth hormones again! She's also bringing up bile/meds lately so we went to see her surgeon who thinks her fundoplication has come undone. She has to have a barium test & x ray soon to check, if it has then it will require another operation to fix it. Shes also finally been referred to physio for her feet. PM turns 3 in 2 months as well. The time sure does fly by! E will be starting school in September & turns 5 in oct!
Well I think that's everything really. One thing after another as usual lol.

Merry Christmas!

Thought I'd best write a post before Christmas and let everyone know what's been happening, if they are interested! The E Coli infection E had eventually went away but it took almost 5 weeks and 2 courses of antibiotics. It made her so poorly, she missed so much nursery as well. We thought after a fortnight she was doing really well until the infection came back, giving her headaches so the hospital gave her stronger dose of antibiotics this time. She was poorly for her 4th birthday, we had to cancel her party. Would have been her 1st ever one as well. Family came to visit though but she was miserable bless her. 2 days after that she was back in hospital. Apart from that and now a cold she seems loads better and is getting excited about Christmas! She loves all the displays in shops and keeps singing "When Santa Got Stuck Up The Chimney" which she's been learning at nursery. Luckily PM has been doing much better than her big sister. Again she's full of cold and 2 weeks ago had a trouble cough and ear infection which soon cleared up after a week of antibiotics from her GP. Luckily no hospital trips needed for her. Touch wood! She's settled into nursery now and enjoys going thankfully. Her speech is coming along at an amazing speed and her Makaton copying skills are awesome! E is finally using the potty sometimes during the day and so far only 1 accident at nursery as she couldn't get her tights off quick enough bless her. PM shocked us today to by doing her 1st ever wee in the potty! Girls will be seeing their eye specialist soon too so hopefully no bad news will come of that although they sad last time E's lazy eye was much worse than PM's so she might get a new prescription for stronger glasses in a few weeks. Don't think there is much more to say really at this moment. Just got my fingers crossed the girls stay out of hospital over Christmas and stay pretty healthy.

When It Rains, It Pours.

That's our new motto for this family as when something happens it seems a catalyst for more stuff to happen. Since my last post E has spent a week in hospital with E Coli in her blood. She went in Friday night after being sent home from nursery. Temperature was 39.9 but after some Calpol she seemed to brighten up. A few hours later during bath time she started to shake, go pale and blue and then said she saw monsters. So we rang the renal ward and was told to bring her in for a check up. Few hours later she had been admitted, had bloods done and a urine sample. Find out later she had a kidney & urine infection which caused the E Coli to grow in her blood along with the infections and make her poorly. Took her in in time it seems as they said if it spread she could have ended up in ICU. Luckily she responded well to very strong IV antibiotics and had to have IV fluids too due to being dehydrated. She was in hospital for a week, took them a few days to get her temperate under control! Glad she's home now as its her birthday on Monday and would have hated for her to be in hospital for that. We had to cancel her party though as wasn't too sure when she would be discharged. During her stay she went for a scan to check her kidneys, liver etc. They noticed again she has an enlarged spleen and now they want yearly checks on it so they can keep an eye on it. They aren't sure if its Cystinosis related. In 4 ish months time she has to go back for an x ray on her Kidneys after being injected with some special dye to see if the infections have left any scarring on her kidneys. Hopefully not! She has to stay on the antibiotics for another 8 days but she can have them orally now, well via her G tube and she's having 2 bolous feeds a day. She even ate some yogurt in hospital. Seems staying there helped her achieve a few things as she also started drinking from a cup! The night she came home she did her 1st ever wee in her potty too. So proud of her. E's back in clinic in 3 weeks to double check the infections have gone and that she's doing okay. PM is doing pretty well, being a bit naughty lately but think she felt a little left out with all the attention Emily was getting. Soon as we where all home it was back to normal and they where fighting as usual! Think she's glad her big sister is back though, regardless of the fighting. Regards to other things, the girls Grandma was diagnosed with lung cancer and has been in hospital now for about 3 weeks and their half uncle died recently after a major heart attack and our over is broke! Anyway, have to go as the girls want to play outside so best go grab them lots of warm clothes as its rather cold today.

All's Well...

Girls recent trip the Renal Clinic bought more good news for them both. Both again had grown and gained weight. Both had pretty good Blood Pressure too. Love it when we go and its all good news. Bloods from the last appointment all came back too so no worries there. Except the results this time showed E's potassium level was a little low so now she's up to 18mls of KCL. PM is being referred to Physio soon as she still trips over her own feet a lot and her feet turn inwards. She's currently now doing her allocated 3hrs a day at nursery, she's still not too sure about it all but after a 10-15mins she's not too bad and enjoys playing outside. Nothing new there! E turns 4 in less than 2 weeks. Cant believe how fast the time is going lately. She's come along so well in the past 6 mths with her speech and other things. She loves dancing and singing, esp to Gangnam Style which is her new fave song lately. She's having a party for her birthday, I'm nervous no one will turn up and its the 1st party I've thrown so no idea what kind of things to do yet! Christmas shopping is all under way, most of the stuff that's been bought has already been wrapped so the girls cant see anything haha. Not much else to report I don't think. We are all full of cold at the moment but girls are just getting on with it, poor PM has almost lost her voice though. Sounds good but its sad at the same time bless her! Back to clinic in 6 weeks and eye clinic beginning of November :)

Late Update!

Well sorry it's been a while since updating. My laptop broke but have managed to get the app on my iPod touch! The girls are doing excellent lately, E was admitted a few weeks ago being dehydrated and generally unwell. She spent 4 days in bless her and didn't get woken every morning at 6am for bloods which we both appreciated. PM still isn't growing much and is full of cold again but apart from that she's doing well. She's started visits at nursery now and is enjoying them. Her speech is coming along beautifully as well. E is still loving nursery to and her speech is amazing now, she has loads of friends there :) No new medicines lately which is great but both their Cystagon dosages where upped, unfortunately neither of them coped well with the increase even when starting slowly. But apart from that everything is going well lately and my girls are still happy and relatively healthy! Clinic again in Oct to make sure and a few other appointments in between. PM does so well with her glasses she doesn't even settle at night unless she falls asleep wearing them! E lost hers somewhere but she should have a new pair next week hopefully. Girls now have another pet rabbit and 2 little hamsters. They love animals :)

May Update.

Well clinic visits are always fun. I say this totally in a sarcastic manner. Last clinic visit last week wasnt too bad though. Except again with the high BP's and the crazy old fashioned way of finding one and the use of a doppler on little feet. The Dr says Cystinosis patients dont usually suffer from high BP anyway so I dont get the point of all the big whoo haa about it that takes like 30 minutes but never mind. Girls where happy after being given a sticker. Surprising news that E now weighs 30lb!! Seems to have come on from no where, such a hugh improvement and an awesome milestone. She's also grown a few cms. PM the same, although not so heavy but gained weight and a bit of height. E's Cystine level was pretty high so an increase there in her medicine, now on 200mg every 6hrs. PM's was SUPER low, like the lowest its ever been we where so amazed. It was like she didnt have Cystinosis for a few seconds. Everything else came back pretty good as well. PM's x ray showed no signs of rickets in her legs/feet and they'll be checking her hips soon, mainly due to the way she walks/trips over a lot. Rickets is very common in children with Cystinosis before anyone comments about me letting them out in the sun or whatever, do some research! E's managing fab with her insoles, she has a review in June on them. Both doing well with their glasses but its a struggle keeping them on some days and PM has a habit of hiding hers, in pretty good places for an almost 2 year old. Took us 3 days to find them last week. They also started E on a new medicine, bringing her daily total to a whopping 10! Im sure over the years we'll beat that record at some point. PM still on 8, still a lot but normal to us. PM's going back to eye clinic soon, she seems to be having problems with her left eye so it needs reviewing. She turns 2 in 15 days!! Cant believe how fast that has flown by, she doesnt look big enough to be 2. Few more months and she'll be at nursery with her big sister :) PM's speech/Makaton is coming along nicely, she's trying to communicate more and picks it up so fast! She's a clever little monster. I never give these blogs good titles, never know what to put. So sorry its a bit lame. Not many people read it anyway so not too bothered lol. But I think thats everything at the moment. All is well that ends well as they say!

Highlighting Cystinosis & Being Honest.

A few weeks ago I recieved a message from a publishing company, interested in my girls story and available to get the story out there and highlight Cystinosis. Didnt think it would do much but any awareness is better than none, well to my surprise we where featured in The Sun newspaper and online via The Sun and Daily Mail. Lovely articles as well with some gorgeous pics of my girls together. Have also been told a few TV programmes might be interested but we are still waiting to hear back. While its nice they highlight the disease and get some awareness out there they never fully understand what Cystinosis is and what it entails 24/7. Most of the time no one even knows the girls are sick and that in itself can be a blessing, they can lead normal lives and be happy little girls. But sometimes, it all gets too much. For me, for them. Medicines every 6hrs including during the night. Near on impossible to do eye drops hourly during the day and preparing the machines/milk feeds over night, lots of nappy changes due to fluid intake. The trips to hospital, the blood tests, the x rays, the tears and tantrums. E getting so upset when retching from afternoon meds or saying her legs hurt or her eyes hurt or asking for the blinds to be closed as the sun is too bright at only 7/8am in the morning. PM always full of cold, sneezing, coughing, not sleeping well. No offence to anyone but until you live it day in/day out they have no idea of what goes on or seem to think these children are going to grow out of it all. If only it was that simple and easy! This disease has NO cure. It takes children away for their parents, loved ones away from families. It can make you blind. The medicine makes you even more sick, gives off funny smells and some children are bullied because of this which makes me very angry. Makes some children not want to take their life saving mediciation due to bullies. Some children have been home schooled due to this as well. I know some people with Cystinosis who are in there 30's and seen as miracles, told they wouldnt live passed their 10th birthdays but still fighting strong, still in some ways suffering from stuff like muslce wasting but healthy in their own little ways too. Some are mothers which again is a miracle, its very hard for anyone with Cystinosis to be a mother and so very dangerous for both mother and child during pregnancy. Its hard knowing your child is likely to be bullied, hard seeing them play with dolls and know deep down they might never get the chance to be a mother and you a Grandmother. Knowing there's a big chance you are going to outlive your child(ren). Know if your child doesnt keep taking medicines kidney failure comes much quicker. A transplant DOESNT cure these people. Cystinosis then affects other organs like the brain, liver etc. If only it was as simple as outgrowing it or getting a kidney transplant. There's simple things in life I cant enjoy like other mums, having your child climb into your bed when he/she is upset or scared or poorly. Mine cant do that due to being attached to milk feeds all night. I dont normally blog such personal stuff but feel like getting some of this out there today. I am so blessed to be a mother I know this, especially to 2 amazing little girls who make my life worth living. I wish Cystinosis didnt exist, I had never even heard of it until E was diagnosed and by then I was 7mths pregnant with PM. Ive lost count of the amount of times Ive had to literally pin them down for nasal tube passings or blood tests or G tube changes, lost count of all the hospital stays. The Drs and Nurses know us all, like a little family in a way. My girls are only young so I know there is so much more to come but we are also hopefull for new medicines, especially the delayed release Cystagon which will only need doing every 12hrs instead of 6 but there's the added worry of the NHS getting hold of it or wanting to. Cystinosis affects people differently. Some seem to have no other underlying issues whereas others do. So in some ways some suffer more than others. I dont like to use the word suffer but when your almost 4 year old cant even walk down the street without being in pain then its needed. E is getting a disablility pram in a few weeks as she's outgrown her normal one. Both girls are having hearing tests done soon as well and we are at hospital again on Thursday for Renal Clinic. Sometimes you feel like you live at the hospital or might as well do. It would be much easier!

Good few weeks.

Well the recent clinic appointment went pretty well. Both girls levels are looking excellent, G tube sites looking good and both had gained some weight and a little bit of height. E is now 13.5kg and 88.3cm while PM is 9.3kg and 77.6cm. PM had an eye test recently due to a squint, turns out she is actually very long sighted so she now wears glasses. First few days she wouldnt keep them on but now she normally only takes them off when she's tired or frustrated. We also mentioned about her walking as she tends to limp and stumbles a lot. Dr had a look and decided to send her for an x ray. We are just waiting for any news now really. E is still currentyl loving nursery and had adapted so well to the 3+ room, her speech is just amazing now. PM is due to start in September. She is currently seeing a portage worker who comes to the house to play with her, this has helped her confidence loads and she is also now trying to say words. We use a lot of Makaton which both girls have taken to, both superstars at it. Yes Mr Tumble is a firm favourite in our house, we all enjoy watching it and learning new signs. Both the girls due another eye test soon to see if the crystals in their eyes have improved, got worse or no chance. PM's had improved last time, unfortunately no change for E. She still hates the sun shining in her eyes so just had to stock up on sunglasses for her. PM is now in a big girls bed bless her, I didnt want her to at 1st as she's still so small but she loves it and sleeps loads better in her bed. She looks super comfy in it as well!

We visit the renal clinic now every 6 weeks. Just routine stuff mainly and never really expect anything eventful to happen. Until our last appointment there!
All was going normal until they checked PM's blood pressure. High 2 times so they said come back after you've seen the Dr. Anyway. we're in seeing the Dr who now thinks E has asthma and was given an inhaler and they think PM might also develop it as well. Not the easiest thing at 1st trying to get a 3 year old to take her inhaler through a mask! E had to have another chest x ray and she's getting special insoles for her feet soon as her ankles are going in but her knock knees don't look any worse, bad thing is they don't look any better either.
Then they test PM's BP again and still high so we get sent up the ward for manual testing. Cut along story short it was done 8 times until they finally got a lower reading. They even used a Doppler twice on her little foot to listen for the heartbeat! We had to get her asleep before the last test but luckily that didn't take long as she was shattered bless her.
It was quite an eventful visit to say the least. Then the pharmacy didn't give us a mask for E's inhaler so we had to go through the GP and they are pretty useless most of the time but alas, they prescribed her one and gave us another tube with teddy bears on!
She's fine taking it during the day when wide awake but when she's coughing at night and half asleep she isn't keen at all. Think she panics at the thought of having a mask put over her face. I know I would!
PM been referred to eye clinic about her lazy eye. E's back at the bone clinic to get her insoles fitted soon then again in 6mths for another review. PM might be joining her as she walks with a limp and to say she's been walking for a few mths now she is still very unsteady and trips a lot.
E had lost some more weight and PM was about the same so growth hormones still in the pipeline for PM.

Hello!

Been quite a while since I last posted so thought I best get on with it! We had a lovely Christmas although there was a day or 2 when we thought we would be in hospital celebrating. Both girls where poorly week before and E was admitted, they tried with PM to but the ward was pretty full. Took them 6hrs to get E a bed!
Her potassium was low at 1st then when that was fixed her phosphate went low so she was on drips most of the time. They both where given anti biotics and after a few days where much better! They do not manage well with colds at all, it knocks them for 6.
Both of them have also lost a bit of weight and we where having problems with E's button hurting her, she wouldnt scream and cry if you went anywhere near her belly. It was a nightmare getting her on and off her milk and doing meds. The community nurses didnt want to change it 'cos of "its costly" but we couldnt leave her in agony so we decided to change it ourselves. Its been better since although still a little sore for her but bet its been rubbing and thats what has caused the pain.
Missed PM's endocrine (growth) appointment, we didnt even recieve a letter so was pretty pissed about that. Been waiting for months and now we have to go on the waiting list again! Also missed her Speech & Language appointment, another one we didnt know about until a letter came saying we had missed it! Really annoyed me as well.
E goes back to orthapedics in Feb to see if her knees have got any worse or better. She has to go every 6 months for that now. PM still hasnt really grown bless her, she's 20 mths old and still fits in 6-9mth clothes! Only thing growing on E is her bloated belly lol.
PM has lazy eye as well so we are awaiting an appointment to see her special optician. Hopefully it will correct it self or she might need a patch over it for a while. She might also be starting nursery in September! She's become a very cheeky madame and has started hitting E back if E hits her!
Apart from that everything has been pretty good. Looking forward to the warmer weather when we can get out more and do things! Oh and MrD has finally started his driving lessons, so hopefully maybe by Christmas we might be driving ourselves rather than relying on Grandad! Yaaay.

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

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 :)