About Me
Hey everyone! I’m Meagan and welcome to my blog. I am 33 years old and I love cooking (but more importantly, eating). Other things I love to do are travel, read, and do yoga. I have something called PKU that affects what I can and can’t eat. I’ve been on a low-protein diet my whole life and it has always been an adventure trying to find things to eat. Restaurants are particularly difficult, but sometimes eating at home just gets too boring. Fortunately, I love cooking. I have tried a lot of recipes and a lot of weird food combinations over the years. On this blog, I want to share my PKU friendly recipes and food tips to help out other PKUers. I hope that any recipes, tips, or stories I share are helpful for all of you out there with PKU or for parents of children with PKU.
What is PKU?
PKU, which stands for phenylketonuria, is a rare genetic disorder that affects a person’s ability to metabolize phenylalanine. Phenylalanine is an amino acid in protein. The enzyme in my body that converts phenylalanine into tyrosine doesn’t work right. So when too much phenylalanine gets into the brain, brain damage can occur. As an infant or child, this is much more severe and can lead to intellectual disabilities (previously known as mental retardation), especially if undiagnosed or if not following the low-protein diet. As an adult, physical brain damage is less likely, however being off diet can impair brain functioning. Most notably, it affects executive functioning. Many PKUers who have gone off diet at some point in their life also describe something called “brain fog,” which is basically when your thinking isn’t as clear and straightforward as it could be. When older adults go off diet, it can also be reflected in their behavior. Common symptoms include irritability, fatigue, impaired memory, and many others. These symptoms appear because phenylalanine blood levels have increased in the brain.
PKU is diagnosed through the newborn screening test when a child is born. Once diagnosed, the child is placed on a low-protein diet. PKUers also drink formula, which is how they get their protein. Everyone needs protein but PKUers cannot get it from food like everyone else. There are special companies that make formula and low-protein food alternatives that allow PKUers to (1) get protein in a healthy way through their formula, and (2) have low-protein options for typically higher-protein food products like meat, dairy, and wheat alternatives.
PKU can be treated by following a low-protein diet with formula, as I mentioned previously. Recently, there are other options for helping treat PKU such as Kuvan and PegPal (or Palynziq). Kuvan comes in a pill or powder format that, if a PKUer is a responder, will allow that person to increase the amount of protein that they can eat in a day, Typically, people on Kuvan still need to drink their formula. PegPal I’m a little less familiar with but here’s what I know: It only just became FDA approved this past summer (2018) and is administered with an injection. Apparently, this has allowed people with PKU to drastically increase their protein level intakes as well as decrease the amount of formula they need to drink. That’s about all I know about PegPal.
Check out more information about PKU at NPKUA website or the PKU News website. If you want to know more about how Kuvan works click here, and if you want to learn more about Palynzic click here.
