So it’s been awhile since I posted an update on Elsie and that’s mainly because she’s been doing really well! Her weight and height have been following the growth curve at about the 15th percentile which is awesome. And for the most part her levels have been really good with the exception of her potassium and most recently her white blood cell (WBC) cystine.
The potassium we have been struggling with for awhile. She’s just a little bit below the normal range but still enough to be a concern so originally our doctor prescribed potassium chloride in addition to the potassium citrate that she was already taking. Right away she started throwing up again, usually before she even finished taking all of the potassium chloride and after a couple weeks it didn’t get much better. So we requested that another combo be tried or that the potassium citrate be increased and our doctor recommended the latter.
At the same time as starting the potassium chloride he also prescribed spironolactone, a diuretic. You may be thinking why would a diuretic be prescribed for a someone with Fanconi’s Syndrome? They pee enough already! We thought the same thing but this one is supposedly a low diuretic and also happens to be potassium saving. These changes helped bring her level up a bit, but still not enough. After our last visit her potassium citrate was again increased and we’re just waiting to hear from the nurse about the results.
Her WBC cystine level came back this week and surprisingly it was at 1.20. The optimal range is below 1 and just three months ago it was 0.13 (which we were so happy about), so it seems strange that it would jump so much higher in such a short time. Our doctor would like to leave her Cystagon dose as is and we are not comfortable with that because a level over 1 will still cause some damage to her cells. He thinks that outside factors may have contributed to the higher result but if that’s the case than we would like to have her retested. Unfortunately this probably won’t happen due to the cost of the test and I am not okay with that. While we’ve been dealing with the potassium levels Elsie has been tested and retested quite a bit so I don’t see why it should be any different for the WBC cystine. I feel that to not retest just because of the cost is a huge disservice to her health care. I sent an email to her renal team about it yesterday but have not heard back so we’ll see what happens.
It’s been awhile since I’ve posted her med schedule so I thought now would be a good time since there’s been a few changes.
1am – Cystagon 175mg
7am – Cystagon 175mg
With breakfast
o Potassium Citrate 8m
o Sodium Phosphate 1ml
o Spironolactone 1.2ml
o Cysteamine Eye Drops 1 drop per eye
1pm – Cystagon 175mg
With lunch
o Potassium Citrate 8ml
o Cysteamine Eye Drops 1 drop per eye
6:30 pm – Omeprazole 3ml
With dinner
o Potassium Citrate 8ml
o Sodium Phosphate 1ml
o Spironolactone 1.2ml
At bedtime
o Somatropin 0.5mg
o Cysteamine Eye Drops 1 drop per eye
7pm – Cystagon 175mg
Although both the potassium and the WBC cystine levels are a big concern at the moment Elsie is still the goofy, crazy, loving girl she’s always been and we’re very happy about that. Lately she’s been a little more on the crazy side but it’s a life with a new baby issue and adjusting to mommy’s time now being spilt. Sometimes it’s nice just to deal with common issues!
Elsinosis: Living with Cystinosis 