Friday, 30 July 2010

living with Zac

As Zac has grown his list of medications gradually grew with him. He started on just a few drugs..... cystamine, potassium, eye drops as the cystinosis affects the eye as well etc.
currently this is Zac's list of medication:
cystamine: to be given every 6 hours 12md, 6pm, 12 md and 6am
cystamine eye drops: 4 times daily
enalapril: 2.5mg daily (to reduce pressure on kidney)
frusemide: 5ml daily (Zac has been retaining excess fluid)
ranitidine: 4ml twice daily (helps protect Zacs stomach from acid)
omeprazole: 30mg daily (also to protect stomach form acid)
These below are given to replace electrolytes lost because of the kidney failure:
potassium chloride: 15ml twice daily
potassium acid phosphate: 5ml twice daily
sodium bicarbonate: 50ml three times daily
other medications he takes:
sodium ferededate (sytron): 10ml twice daily (to treat anaemia)
darbopoetin: 20micrograms weekly (as above)
alfacalcidol: 4nanograms daily (replaces vitamin d)
norditropin simplex injection: 1mg daily (human growth hormone)
levocarnitine: 4ml twice daily (aids muscle strength and growth)
piroxicam: 10mg daily (ease joint pain due to arthritis)
Getting these medications into Zac is almost a 24hour a day job. Our routine starts at 6am when he gets cystamine and some others. We stagger the times of his morning meds as he gets so many. He then gets more at 7am and once he gets up if its a school morning. His school is fantastic and give him his lunchtime medication and also have been trained on using his ng tubes when he has had them and also have been incredibly accommodating with his other needs which have been growing lately.
After school Zac gets more medications when he gets home. Again, before dinner and after dinner. And each night he needs an injection of growth hormone as most cystinosic children don't produce enough to grow normally. Even with this he is noticeably smaller than his peers.
Once in bed Zac is attached to his feeding pump which provides liquid feed over 12 hours. More medications are given at 9pm and finally at midnight. A good nights' sleep for us is 5 ½ hours. Usually sleep is interrupted most nights with either his pump alarming or him running out of water to drink.
With cystinosis comes many clinic visits!!!!! over the years our clinic list has grown like his medications list. Currently Zac attends:
renal clinic approx every 6-8 weeks, sees consultant, dietician, pharmacist
orthotic, 6 monthly
optician, 6 monthly
rheumatology, 6 monthly
orthotic dept 6 monthly
other people Zac sees regularly include: physiotherapists, psychologists, family psychiatry liaison team, surgical clinic, respiratory.
In the 9 years since Zac was diagnosed he has also had a few (relatively minor) surgeries. First was to site his original PEG feeding tube. This was replaced with a Mick key feeding button after a year. He has had a Nissian fundoplication which prevents him from being able to vomit. Mick key button removed and replaced with various tubes. Tonsil and adanoidectomy as he had sleep apnoea. Feeding tube removed and closed as he was rejecting his button. New site established after 12 months.
We have been rather guilty of not telling people just how much goes into caring for Zac. Those who have cystinosic children will probably agree. I think we tend to let – even close family and friends – in to a certain degree..... but we keep a huge part closed off. Why? I don't know.
Its hard looking after Zac a lot of the time. There are many nights when you lie awake and all you can do is think about everything related to life with a cystinosic child.... the things he wont be able to do, things he wont even get the chance to do, the dreams which will never happen, and – dare I admit it, there are even times when I have thought about how inconvenient it is, how jealous I get of people who get 8 hours of sleep... how my life is harder...... and then the guilt hits for feeling these things. Wondering if he will go home after a clinic visit or be admitted to the ward, that wait after getting home for blood results every six weeks to see if we need to change meds or even bring him in to the ward.... Life can be hard.
But life can be so amazingly fulfilling, and humbling, and wonderful. Those tiny things. Like watching him laugh with that infectious laugh. When he manages to do something for the first time. When he achieves something he has struggled with. When you watch him laughing with the nurses an hour after theatre with his lines still in, on oxygen and insisting he wants to walk to the toilet with a cannula in his foot!!!
These are the times I am so damned proud of my son!
And I am proud to be his mum...


  1. Cant belive all the meds he has hun, so many! Some of them very familiar to lol. Potassium chloride at 15mls! Emily has that to but only 3mls lol.
    Following you3 blog now hun :) Love from another mummy who is very proud of her children :P xx

  2. Hey just found your blog. It's interesting to read it from a parents point of view.
    If I wrote a blog, my Cystinosis wouldn't even get a mention! As I've gotten older, it's gotten easier to control and deal with. After my transplant the meds are a lot easier and way less!!
    As I say to all parents, don't worry about the future too much - my parents did and a lot of it was needless. You can live with it and I have a pretty normal life! Obviously we are all different, but I know a few adults with it and it doesn't hold us back.
    Keep up the fab work!! Holly (23) xx