This is all the medication that Elsie has been prescribed and now takes daily since being diagnosed with cystinosis. From left to right it’s Cystagon, calcium, cysteamine eye drops, potassium and sodium. Seems like a lot for a little toddler to take doesn’t it? I have to say though we have been really impressed with Elsie so far. Obviously she puts up a fight at the beginning but she realizes quickly that we’re not going to let up and often now reluctantly accepts it.
We’ve been giving her the eye drops for 3 weeks now and they are probably still her least favourite. It is a cysteamine solution that removes the crystals from her corneas and takes the excess cystine away. We’ve been told she needs to take 1 drop per eye twice a day, which is nice because it seems most children are taking them 8 to 10 times a day so for now I’m happy with the lower dosage. Often it’s either my husband or I giving her these drops alone which can be a challenge. The technique I use I found on Livestrong where I lay Elsie on the floor in between my legs with her head closest to me and her arms tucked underneath my legs so she can’t wipe the drops away before they get into her eyes. When she sees the dropper coming she closes her eyes as tight as she can so I put a drop in the corner of each eye, near the tear duct (but not too close so the duct doesn’t cry the drops out), and then gently try to open her eyelids a bit so the drops roll in. Once I’m satisfied the drops have made their way into the eye I say all done, quickly pick her up to sitting in my lap and we have a cuddle.
The minerals, calcium, potassium and sodium, we’ve been doing for about 2 weeks now and there’s good days and bad days. These are needed to replace what she is losing in her urine due to Fanconi’s Syndrome. For the most part after the initial crying and fussing she sees we’re not going to stop and will open her mouth and take it, but not always. Sometimes we just have to restrain her and drop a bit in every time she opens her mouth. All 3 need to be given twice a day, the sodium and potassium with meals and the calcium in between meals. The technique I use for these is to sit her in my lap with her closest arm wrapped behind my back and then I can grab her other arm with my hand that is around her back. When she sees the syringe coming she’ll usually try to throw herself back and start crying. She’s learned that we give her the medication when her mouth is open so now cries with her mouth clamped shut, shaking her head back and forth which is quite funny to watch. If it’s the calcium we usually just have to get a bit in her mouth so she can taste it, then she relaxes and is usually content to suck on the syringe while we slowly dispense it in. This is because the calcium tastes pretty sweet. The sodium and potassium are more salty tasting, which she doesn’t overly enjoy but sometimes she’ll do the same and suck on the syringe.
Finally there’s Cystagon. This is the one she needs to take every 6 hours and it takes away the excess cystine in her cells. We’ve only been giving it to her for 3 days now and it goes much the same as the minerals. It comes in caps that we need to break apart and dissolve in water and yes it doesn’t taste or smell very pleasant but so far she doesn’t react any different than if it was sodium so that’s a plus. Right now we need to slowly increase the amount she takes every week until she’s up to their desired dosage which should take about 4 weeks. The schedule we decided on was 7am, 1pm, 7pm and 1am and I was the most concerned about the 1am dosage and how that would affect her sleep. So far it hasn’t been that bad. She wakes up, takes it, we put her back in her crib and she falls right back asleep. Now we’ve only done this twice so I’ll let you know if it continues to go this well but we’ve been super happy with it so far!
We’ve been told the common side effects of Cystagon are nausea, vomiting, diarrhea, loss of appetite and lethargy. So far it just seems to be vomiting that is affecting her. I’m hoping that this will just be temporary while her body gets used to the medication but only time will tell. She threw up once yesterday in the afternoon and once this morning so my plan is to post on the Facebook support groups I just joined (check out my resources page for their links) and see if any parents have some natural tips to reduce vomiting. As for the loss of appetite she never really ate much to begin with so it doesn’t seem like that’s decreased too much these past few days.
If you’re curious here is Elsie’s current medication schedule:
1am – Cystagon
o Sodium – 0.5ml
o Potassium – 1.5ml
7am – Cystagon
7:30am – Eye Drops – 1 drop per eye
9 to 10ish – Calcium 5ml
1pm – Cystagon
3 to 4ish – Calcium 5ml
o Sodium – 0.5ml
o Potassium – 1.5ml
7pm – Cystagon
7:30pm – Eye Drops – 1 drop per eye
I haven’t added the dosage of Cystagon as right now that will be changing week to week but once her levels are balanced I’ll post her schedule again. I’d be lying if I said this schedule doesn’t get overwhelming at times but I’ve found that once you’re doing it and you take it one dosage at a time it’s really not that bad. Yes it sucks that this will be Elsie’s life but in the long run it will just be one small part and this routine will allow her to continue to be the active happy girl she’s always been.