A mean o’ Acid

As I have blogged about before, my children have an inborn amino acid deficiency. They have a gene defect so that they do not make an enzyme that breaks proteins they eat down into amino acids, and without this particular one, well, you get Pearlsky and her brother.

I have been buying the amino acid, 2 kilos (almost 5 lbs.) a month from Carlson Labs for about 15 years, and they abruptly discontinued it, without notice. I called to re-order and they said, sorry, we don’t sell it anymore. They did sell me the last bit they had at an inflated price. When I politely asked them who their supplier was, they did not respond. So I am here to say, Carlson Labs is one business to stay away from.

I searched the internet and found suppliers, almost all in China, all wanting me to buy at least a pallet worth (75 or 100 Kilos). The two suppliers in the US wanted three to four times the money I was paying ($350 per month). Finally, a local compounding pharmacy said they could help me. I recently picked up 2 kilos from them, for $300.

Sounds great, right? Without this amino acid, my kids would not survive. Without the right amount, they have seizures and other effects.

I open the new container … and it looks different. Here, take a look:

On the left, the old stuff. On the right, the new stuff. One is crystalline, the other powdery. One I can purchase, one I cannot.

Medicines and such are ideally given by weight. I know from our own research (on her) and the small bit of literature out there (remember, she and her brother are the first known with the problem) (we now know two other kids in the US, a dozen in Europe) exactly how much amino acid she should get, by weight … 24 grams per day. I use a specific scoop to measure this out every morning … the old crystalline stuff needed six scoops, the new needs seven and a half to make 24 grams. The old stuff, when dissolved in water was clear … the new stuff is yellowish. The old stuff tasted bland, like boiled water, the new stuff tastes, well, awful.

Now what? I cannot get any information from the evil Carlson Labs, one thing I really really need to know is the assay, a test that will tell me how pure the amino acid is. I know the new stuff is 99.7% pure, it came with its assay, and I also know its origin is China!

So, where does this leave us? Let us assume that the old stuff was not pharmaceutical grade pure (as the new stuff is). That may explain the differences, but then, what else was in there? Carlson Labs ain’t talking. If it was not pure, then giving 24 grams of the new pure stuff will be a lot more of the amino acid than giving 24 grams of the old stuff because 24 grams of the old stuff was x grams of the amino acid and x grams of unknown.

The saving grace (who is grace and who is she saving?) is that the literature most likely refers to (in Pearlsky’s case) 24 grams of pharmaceutical grade amino acid, hence now she would be getting a full dose (which is actually a guess in itself) as opposed to the low dose she was getting before.

So far, so good. She has been on the new stuff about a week, and we are watching very carefully. Her brother will start next week.

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Me? Thanks for asking. I still have a bad case of sciatica which, by the way, is NOT a diagnosis! I always thought it was, but no, it is a set of symptoms, which in my case, hurt like, well you remember, they hurt. They arise from something messing with one of five nerve roots at the lower part of your spine (lumbar 4 on me). I just started driving again, only around the neighborhood, and I still walk with a hefty limp. The pain does not wake me up at night anymore and it has actually moved up from my foot, to my shin, then up to my thigh, and now mostly in my upper thigh and butt. I am told this is good. Wonderful.

Time to prepare for this week’s IEP …