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 :)

Ugh.

Well today we took E & P to the hospital. It was an appt just for E to have some bloods taken but it turned into much more. We arrived a few minutes before 12 and E's usual Dr saw us waiting and we all got talking. We started to mention about P not gaining much weight and last 6-8 hours on just 2-4oz of milk and they agreed it wasn't right. They weighed her (10lb 8oz)and also noticed she hadn't gained much weight. After weighing she also had some bloods taken which she wasn't pleased about. Then the dietitian, us & the Dr agreed P having a nasal tube would benefit her and was needed :(
She goes in on Wednesday to have it put down and will have to be in for a few days to see of she tolerates it. Luckily we wont be in a week like we where with E's as we know how to work the pumps, unless she doesn't tolerate it.
I was so gutted but know it will be for the best before her weight starts to decline rapidly like E's did.

Meds etc.

Princess E's current meds are -
4ml of Cystagon every 6 hours.
Eye drops called Cysteamine every 2 hours while awake.
10 drops of Alfacalcidol in water once a day.
3 Phosphate Sandoz tablets 3 times a day diluted in water.
3mls of Ranitdine twice a day.
4mls of Potassium Chloride 3 times a day.
2mls of Potassium Citrate 3 times a day.

At night when E falls asleep she is then hooked up to a milk feed. Taking 500mls at a rate of 70mls an hour. During the night she also has 2-3 beakers of water in her cot which are refilled up to 3 times as she drinks loads. During the day the water intake is the same.

G-Tube.

Princess E has been in hospital over the past 9 days, she finally came home yesterday after having her nasal tube removed and a G tube fitted & a fundo put in place (means she can never vomit). She went down at 9am and didn't return until half 2,longest hours of my life. She was meant to only be in for 7 days but her tummy got quite swollen so feeding was delayed. The hospital stay wasn't too bad, daddy stayed most nights as I was looking after baby P but I stayed a few nights. She had bloods taken almost every day which broke my heart, within a few days of getting there she hated anyone who came near her and would throw a massive tantrum. Broke my heart. Many times I just wanted to take her home!
She is very happy to be home, she loved being back in her own cot last night & has enjoyed being back with lil sis and playing with all her toys & watching Playhouse Disney. The house hasn't been the same without her, far too quiet! Glad she is home now though.

Bad News.

I'm feeling so numb right now. My heart is aching, my eyes hurt from crying. Poppy-Mae has just been diagnosed with Cystinosis.
I'm devastated. Gutted. Heartbroken. Angry and a million other things.I've gone here to vent and whatever else comes out. After losing my placenta they took bloods from P last week. We received a call this afternoon to clarify she does have it. Her level is alot higher than what Emily's was but at least they have caught it early and they started her on the Cystagon today - 1ml once a day then slowly build it up over a few weeks.
I'm so angry with the World right now. I HATE it! I was hoping she wouldn't have it although her dad was convinced she did, he was right. They took bloods again today to check her Electrolyte levels and to start supplements if needed but they doubt she'll need any yet as she is only 7 & a half weeks old. They want her & E at clinic next week for more bloods again. I'll have to get braver now, I always go out the room when bloods are taken but I best get use to it as its going to be a regular occurrence for them both. I just .. I cant think about anything else right now! 1 in 4chance and she gets it. I swear our family has just been cursed since me & Darren got together. I keep thinking I'm going to wake anytime soon and realise the past 3 years have just been so extremely long bad dream.
I wouldn't change my girls for the World though. They are so special to me and this just makes them extra special. In a way at least they can find comfort in each other when they are older as they'll both know and understand how the other one is feeling. Health Visitor is giving us loads of support thankfully as are other people and luckily I have quite a few people on Facebook who know what we are going through.
I really am heartbroken. I feel so sick to.
But ... long as my girls keep smiling I'll smile with them!

Eye Test.

Well today E had to have another eye test, every few months she needs them now. So she's due back in 2 months.
I stayed at home with Baby P while Darren took her, her appt was at 8:45 and we wouldn't have all been ready before 8am when his dad came to take her, she didn't even get chance to have any breakfast until she got home lol.
I thought they would have been ages as last time we where at the hospital for 3 hrs, they where so understaffed and missing equipment it was a joke! But they returned home just after half 9 :)
E still has the crystals in her eyes, they haven't got worse but it hasn't improved either unfortunately. The optician reminded us to keep up with her drops because if we don't she will go blind & he said at this age if she went blind they wouldn't be able to do anything about it :( We manage the drops at least once a day even though they should be done every few hrs, unfortunately it's still a bit hectic here with a 5 week old baby to look after to and it takes the 2 of us to do E's drops as she doesn't like them and fights it. Now the baby is sleeping more & more settled on her new milk we should be able to do them when they are needed.
The last I thing I want is my beautiful Princess going blind! She's been through far too much already.
Still no results on P yet either... E's due back at hospital at the end of July so if we haven't had any by then we'll ask her Doctor to do a test on P instead. I just hate waiting. I lie in bed every night worrying about it!
I'm meeting some fab people through Facebook though who also have been affected by Cystinosis and I find them a big help. They know EXACTLY what I'm going through and it's comforting :) I hope to meet some of them at some point, esp the ones with children so E can meet all the other special little boys & girls like her.