PKU treatments are something of a point of discussion; while there are a series of products in the works that claim they’ll revolutionize the way people handle their PKU, most of those are either stuck as speculative gestures at the moment, and/or are simply too far off to be setting our sights on quite yet. When it comes to actual treatments there’s one method that’s tried and true: staying on diet.
What is the PKU diet?
Typically you’ll hear the phrase “staying on diet” thrown around in this bustling little community of ours; for those of you that are new to the world of phenylketonuria, the PKU diet is one that emphasizes limiting foods that contain the amino acid phenylalanine (PHE). This means avoiding things that are high in protein such as milk and cheese, beans, chicken, beef, pork, fish, eggs — the list goes on and on.
So while staying on diet may seem like a concept many would be familiar with, this isn’t at all like most diets where skipping a period of time and cheating before hopping back onto the wagon with a new resolve to stick to it this time is exactly acceptable. Where straying off the path of a regular diet may leave you struggling to squeeze into your jeans, going off of a PKU diet can ultimately potentially lead to mental illness, psychological disorders, and neurological deterioration. The stakes are, inarguably, just a little bit higher in this case.
It’s all about the PHE
Typically one of the first steps towards establishing a PKU regimen is visiting a metabolic center that specializes in PKU to help establish what your PHE tolerance is via blood tests. Different people can tolerate different amounts of PHE, so there isn’t exactly a one-size-fits-all solution as far as how much somebody that’s diagnosed is allowed to have.
With that being said, its generally safe to assume that your intolerance will be such that you’ll have some difficulty taking in the required amount of protein your body needs in a day. Protein however, much like most other nutrients, still remains just as much an important aspect of one’s diet whether they can properly digest it or not. A lack of protein can act as a nutritional deficiency, which can leave you feeling weak and weary. So how do you get your daily dose of protein without actually consuming protein? Well…
Formula: your new best friend
Formula is a catch-all term for a protein supplement (usually delivered in a formula/milk/shake form) that people on a PKU diet typically have throughout their days with their meals. Thankfully formula has a variety of ways to take it such as single serving powder packets, powder cans, ready-to-drink pouches, bars, and more. Flavors have come a long way over the years too, so supplementing with formula doesn’t by any means doom those on diet with the same stale method of delivery for the rest of their lives.
In the case of infants diagnosed with PKU, often times the parents will be prescribed a special infant formula that’s allowed to be mixed with a small amount of breast milk or regular infant formula to ensure the baby receives enough PHE for normal development without crossing the line of providing too much that can ultimately cause harm.
What about a medication?
As it stands, medications for PKU are still by and large under development with the exception of Kuvan. Kuvan is a drug that’s been approved by the FDA that helps the body to break down phenylalanine. While this sounds altogether like a miracle cure on paper, there are numerous reasons why a person may have trouble breaking down PHE; Kuvan is only a supplement that doesn’t do the job in its entirety. That’d be too easy right?
Kuvan is mostly used as a method by which to reduce the amount of PHE in somebody’s blood; since it’s not strong enough on its own to take care of all of a person’s PHE, it’s typically used in tandem with a PKU diet.
Looking to the future
Researchers and scientists are always finding new ways to pick apart and play with amino acids and the body’s methods used to manipulate them for its needs, so it’s no surprise that the PKU community may end up a beneficiary of these tendencies. Science is suggesting all sorts of procedures to help with the disease, from enzyme replacement therapy, to gene therapy that intends to inject new genes into a person’s body that’ll help them break down PHE.
In the mean time the safest option is to focus primarily upon staying on diet. While it’s seldom easy, the results speak for themselves. This is where Fitfunner comes in; Fitfunner is being designed to help individuals with PKU take a look at their daily habits and encourage only the best to help keep them safely on the right path one step at a time. We’re thrilled to have the opportunity to assist in this sort of way, and we can’t wait to begin helping those with PKU to live healthier lives.