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#6200
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arkham
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Hello, I was off the diet for around 30 years, so maybe I can help you out a bit. The symptoms you list probably are PKU related. I have a milder form of PKU, so I do have some ability to break down phe, and I don’t have the very pale complexion – I’m naturally very pale (Norwegian ancestry), and don’t tan really well, but I do tan. I also have fairly dark hair and eyes. However, as I said, I have mild PKU. I was taken off the diet at age 3 (which was standard practice in the US in the ’70s) and just went back on recently. I haven’t had much for negative effects of being off…except for one – which I just learned recently (from Dr. Richard Koch) – I’ve struggled with my weight all my life, and apparently high levels of phe in the blood interfere with fat metabolism (or at least that is Dr. Koch’ speculation), and makes putting on weight easy and losing it hard. Now, many people wonder why I went back on the diet, seeing as I have had virtually no negative effects from being off. My initial reasoning is this: having been born in 1972, and treatment of PKU having begun (at least being universal) less than 10 years earlier, no one really knows what being off-diet long-term can do. I thought that the inconvenience of formula and a lower-phe diet were small prices to pay to avoid problems like brain damage. Recently, I read a statment by Dr. Koch that people with the milder form of PKU (like me) who are off-diet 20-30 years generally start having problems much like those with cPKU do much earlier. “If the person is fortunate and born with only one severe mutation and another that permits partial activity of the enzyme that can convert phenylalanine to tyrosine, the person has a less severe form of the disorder. If the diet is discontinued, that person may live for 20 to 30 years on a normal diet, but eventually will develop one or more of the complications seen in persons off-diet, such as mental illness, IQ decline, seizures, etc. Off-diet blood phenylalanine levels usually are between 15-20 mg/dl ( 900-1200

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