It seems that we have successfully determined what was causing the most recent round of illness and discomfort: A very minor change in Esmé’s medications. This change was so minor that no one even mentioned that it might be significant in any way.
About four weeks ago I went to pick up her anti-convulsant at the pharmacy. This time the med was waiting out at room temperature–I momentarily panicked since for over a year a half we have been filling this prescription as a compound. Like most (all?) compounds, it needs to be refrigerated in order to maintain efficacy. But it turned out that the drug was finally commercially available as a shelf-stable liquid. I was actually pretty excited because it meant that we wouldn’t have to worry about keeping the meds perfectly chilled when we are out of the house. It also meant that I would not need to fill the prescription every two weeks anymore, requiring a new physical script from the doctor each time as the drug is a controlled substance.
We went home and happy dosed Esmé with the same number of milligrams of the medication, at the same times, just as we’ve always done. After a few days we noticed that she seemed a little bit more snowed than usual. We hypothesized that the drug might be metabolized slightly differently due to the new form–resulting in more of the drug being “available” to her body. And since we had seen something similar switching from crushed tablets to a compounded suspention (a difference of just crushing the tablets and suspending them in liquid), it seemed plausable.
So we discussed decreasing the medication slightly to see if she perked up some.
What we didn’t realize for quite some time, however, was that her aggressive behavior, her painful urination, her intermittent fevers, her GI discomfort, her tremors, her irritation…all the symptoms of the last several weeks that we had been struggling to sort out…all of them could be explained by a reaction to the new medication or a reaction to the medication being at toxic levels in her system.
Hoping that maybe she just needed less of the new medication we titrated down the dose a bit further. But within two doses at the new level, Esmé began seizing. She had four full-blown seizures like we haven’t seen in perhaps nine months. The first three clustered about a minute apart. So, we gave her enough to make up for the old dose…and she slept for four hours.
It was pretty clear that she just needed to return to the old compounded medication. And we never would have sorted this out without the input of our community of PCDH19 parents or my hubby’s intuition…
After a few phonecalls to our doctor’s office and an extraordinarily helpful pharmacist at the compounding pharmacy, we were able to get the old version of the medication on Saturday morning, in time for her morning dose. Within two doses we could see a clear difference in her demeanor and her comfort level. After two days back on the old version of the medication, as is typical of her after an illness, she is so excited to be feeling better that she is vocalizing more, she is insistent about exploring, and she wants to stay up all night! She remains a bit more unsteady than before, but that should return soon.
The reaction itself is pretty hard to deal with emotionally. To be giving her something exactly as instructed–something that, mind you, felt like a miracle drug two years ago, and have it harm her…it is awful. To have to continue to dose her with something you know is toxic for her (because withdrawl would be worse) while you wait for the thing you know that works is really terrible.
But worse than all of that is to think that we might not have sorted out this little change as the culprit. That such an apparently tiny little alteration in medication could cause such a marked change in her quality of life, that it might have gone unnoticed as a potential cause while we blamed her kidneys or part of her disorder or the season…it is just unbearable.
…as is the question about how much the “good” version of these meds has robed her of.
I know that had this treatment not been available to us Ezzy would likely be seizing, like she was before, every few days…that was not sustainable for her. This medication has been life-saving for us.
But I do wonder, with more perfect treatment might she be less low-tone, more able to explore, more vocal, more coordinated, more able to concentrate?
Probably. How much so, I don’t know.
It certainly hurts to wonder, though.
It is so amazing that such a little change can have such a deep impact. It could have been something in the solution that makes it shelf-stable. But no matter, hopefully you can continue of the compound without too much difficulty.