Given my background as a mom with two kids with this deficiency, as a well as a board certified family physician, my goal is to make lipoprotein lipase deficiency / Fredrickson's type I hyperlipidemia / Buerger-Gruetz syndrome more understandable and liveable for other families.
Thursday, September 1, 2022
Adventures in Formula!
We had another baby! Welcome, Thomas Theophilus! We were even good about getting his blood checked right when he turned 6 months old - triglycerides of >1575. Man. Our 3rd baby with LPLD! So we started collecting my breastmilk that I pumped at work in large mouth jars and using a ladle to scoop off the fat after a day in the refrigerator and saving that fat in bags to donate to the local milk bank. But it wasn't perfect, there was still plenty of fat in the milk. So then I tried firing up the old Ukrainian milk separator and running hot water through it and... it spilled water eeeeevveeeerryyywheeeere. No bueno. I'm not putting my breastmilk in that! We trouble shooted and trouble shot some more, and figured it wasn't spinning fast enough and so the motor was probably shot. I mean, it was 7 years old and had made a few moves, 2 international, so it's appropriate to retire it finally. We ordered a new motor alone, to try to save some money... and 3 months later we're still trying to get it installed. The bolts are hard to reach! Then it turns out the are metric sized bolts instead of imperial! So after lots of purchases and returns from the hardware store, we finally have it set up and running!!!
And now that he's got fat free milk to drink, Thomas is TEARING THROUGH what I pump AND what I have frozen. Drat. I have 3 months to go with feeding him this way, and I'm not sure we'll make it on breastmilk alone. He loves his solids, don't get me wrong, but we can't fill him on on solids sufficiently to slow down his milk intake. Hmm.
So it's time for me to learn about fat free formulas! There are two that I'm aware of - Nutricia Monogen and Enfaport. They are designed for Long-Chain Fatty Acid Oxidation Disorders (LCFAOD) or LCHAD or chylothorax, which means they have lots of medium chain triglycerides (MCTs) (and less fat in general). It turns out you can request samples:
Monogen sample
Enfaport sample
So, onward to another LPLD adventure!
And I was sobbing last night and snapping at my kids in frustration over the milk separator being a pain to use and my milk supply getting low. So, sorry to be so upbeat in this post - I promise I'm not always!
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Hello Thomas, nice to meet you! I wanted to let you know that you are born into a family with a great mom!
ReplyDeleteI feel your frustration. Hope the fat-free formula will work out for you!