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.

Things are going ok.

Well E was at hospital again recently for her check up and her levels to be checked. She has grown a little again & gained a bit more weight, her Dr is really pleased with her lately and so are we. She's coming along in leaps & bounds at times and its fab!
They have upped her Potassium now to 4mls instead of 3mls. Her phosphate has gone from tablets to 2 thankfully and her drops for her bones have increased from 4 to 8! She is getting loads stronger on her legs and will even walk now if you have hold of just one of her hands, she still cant stand for too long on her own and only a few mins if someone is holding on bless her. She still drinks loads of water during the day and can now say Ta really well. She is going through terrible 2's though I'm sure! The tantrums & screaming are amazing.
She loves her baby sister who is now 19 days old :) Still no results on whether she has Cystinosis but they did say it could take up to 6-8 weeks. Her dad thinks she does have it but I'm not sure, I don't even like to think about it and will cry either way.
E is back at hosp on the 22nd for more bloods just to check the change of dosages is working and if anything else needs changing. Hopefully not but we never know until the results are in.
Picture is E having her meds, thanks to Daddy!

A few steps backwards...

Just a small post to update really.
We had to take the Little Princess to hospital the other day. After a few days of being sick on literally everything she ate or drank her Dr told us to bring her in. So off we trundled. They weighed her, took her BP and took more blood. Everything came back fine. I wasn't pleased she had lost weight again, she's meant to be gaining now not losing. They didn't need to change any of her meds or give her anything else so after 3 hours of waiting around we where sent home and just told to let her drink plenty of water which we do anyway as it's the only thing she will drink.
I sat at home, wondering why she was being sick again all of a sudden after weeks of doing so well. Was it the rice pudding we tried? Was it the cows milk? It couldn't be the yogurts as she was never sick on them in the 1st place and she'd happily eat a million a day if she could.
Then it hit me. At her last appointment they had given her Sytron again, before she had Sodium Chloride (not sure if the dr wrote it on the prescription by accident or not) and it never bothered her. It wasn't until I noticed she was back on the Sytron that she had started to be sick again. I mentioned this to her Dad. He didnt think it was that, he thought it was the rice pudding that had given her a dodgy tummy or her Cystagon. I suggested we stop using the Sytron for a day to see how it went and low and behold she wasn't sick! She has been off it for 2 days now and today she hasn't been sick at all! When we go back to see her specialist I'll have to let them know and see if there is anything else she can have instead to replace it that wont make her sick. She's at the hospital this week so we can talk to the surgeon about the operation to have the button sorted for her tummy and an acid reflux thing that should help her when she is poorly to not be sick, the slightest cough makes her throw up!
I'm hoping we wont have to wait months for the operation date, Ive spoken to a few parents who have said their child has thrived after having the button inserted into the tummy and it really would do Princess some good. She's just a bag of bones lately and its heartbreaking. Seems some weeks she does fab and then others its back to square one. Its going to be a very long, uphill battle is this but I know she can do it. She's one brave, strong, amazing little girl and has coped so well. Better than me at times haha.
She's a super star ^_^

Some Good News!

Well E had her Renal appointment on Tuesday. We weren't expecting much to be honest, just to make sure blood levels where still ok and that her meds where working and that they didn't need adjusting.
They weighed her ... 18lb 10oz! A whole 1lb gain in just 2 weeks!! I couldn't believe it. I think letting her have her yogurts again might be helping that, she loves yogurt. Then they measured her ... 74cms! She had grown 2cms in a fortnight. Someone had stuck her in a grow bag for sure.
Mummy & Daddy where VERY pleased. Dietitian was happy and Dr was happy. Everyone was happy! Even E who usually gets so restless and clingy at these appointments was quite happily playing on the floor and crawling around and smiling. Someone surely had switched my daughter heehee. We missed the call from the Speech Therapist unfortunately so called her back today to arrange an appointment, I'm really hoping it does help with her eating more solid food as she really wants to. She steals it off your plate now lol.
The Dr doesn't want her back for another 4 weeks, which I was surprised at but she was really pleased with E's progress and said unless the bloods she had taken come back with low levels of something she doesn't really need to see her :) By the time she goes back she'll be a big sister & I'll be a mummy to 2 beautiful Princesses!
She's back at hospital though May 26th to see a different Dr about having the Mickey Button in her tummy rather than the nasal tube, have heard it makes a huge difference and alot easier to manage so will be loads better as her nasal tube keeps coming out what with all the cough/colds she gets bless her. Community Nurses are fab though and usually turn up in 2/3 hrs to put her in a new one, which she absolutely hates but I always give her MILLIONS of hugs & kisses afterwards and sometimes she falls asleep on me.
She's been through so much in the past 9ish months and she's so brave. I'm so proud she's my daughter! Glad she is finally gaining some weight after losing so much, she'll be my Little Chunky Monkey again in no time if she carries on gaining 1lb every fortnight :p Here's a recent piccie of her without her tube and with a cheeky look on her face as usual lol.