This is well outside of my range of expertise. We'll see if any more knowledgeable peeps chime in. Sorry to hear about this. It give you a real appreciation for all of the things our bodies do correctly without us having to think about them.
Does anyone have experience training or working with trainees with PKU. He has been treated properly his whole life, and has no mental problems. He is fairly active, or at least able to be. He just graduated from police academy. He just wants to get a bit stronger. His body cannot break down amino acids, so he has to be on a very low protein diet or else he will suffer terrible health defects. I'm just wondering how strong a man who can only consume 8 grams of protein a day can become. The only thing in his diet that is similar to protein is his specially prescribed amino supplements that already cost him 1200 a month. Nice guy that just wants to be stronger to be a better dad and cop.
This is well outside of my range of expertise. We'll see if any more knowledgeable peeps chime in. Sorry to hear about this. It give you a real appreciation for all of the things our bodies do correctly without us having to think about them.
Oh for sure. Something we should all think about it before we use "genetics" as an excuse. I didn't accurately describe what PKU was in the post above. I won't correct it because I am still learning more. Figured I'd take a shot in the dark on here, I know there's a couple of M.D. types.
I do not, however my daughter has PKU. To be precise, it's not a problem with all amino acids - it's a deficiency of the enzyme phenylalanine hydroxylase which synthesizes tyrosine from phenylalanine. The classic PKU patient will have high phenylalanine levels and low tyrosine levels. The formula he consumes daily is essentially an amino acid slurry with the phenylalanine omitted and tyrosine added. Tyrosine is not an essential amino acid for us, it is for them. The natural protein in his diet will provide the little phenylalanine he actually needs.
Elevated phenylalanine levels while the brain and CNS are developing produce irreversible mental retardation. Interestingly, the convention wisdom in years past was for people with PKU to go "off diet" at about age 13 or so, but the resulting high phe levels have been found to contribute to various behavioral problems even in adults and the trend now is for the PKU patients to make the diet a way of life.
Having said all that, there's nothing fragile about these people and his formula will provide the equivalent of a certain amount of protein each day. I guarantee he can get stronger, but may have to progress slower. I'd just run the LP with him and see what he can do.
There you go. These boards prove their worth from time to time.
Thank you Kent Barry. Like I said, I came to realize how incorrect my earlier explanation was. I have learned a lot since then. Thanks for adding more to my knowledge base. I didn't know this existed a week ago.
Outside the specialty of endocrinology, even most doctors know little or nothing about it. My wife had to educate our pediatrician, who had never had a patient with the condition.
If I correctly understand your first post, it seems you're training this individual or considering training him and you have spent a few days educating yourself about his metabolic disorder. If so, you'll make a great coach. Carry on.
To add more to Kent's great explanation, I've got a family member who did PKU clinical work for her entire career, and she just said that PKU has no impact on physical activity (provided you comply with the diet).
Side note: there's also reversible mental retardation, based on diet compliance. That is, if you're off diet for a limited amount of time, you'll lose some mental function, but can regain it by getting back on diet. So, she'd get to know kids she treated over time, and be able to tell how compliant they were with their diet just by having a random conversation with them.
Awesome replies guys! This has been great to get me out of my comfort zone. I usually prescribe a quart to a gallon of milk a day for extra calories and good animal protein. He let me know he couldn't do that, and why. I asked if he could just double up on his pku formula. He informed me that isn't feasible, he already spends over $1000 a month on it.