Posted on 12/17/2009 4:06:36 PM PST by Chickensoup
King George didn't have that. Instead, his problem was with "recycling" heme, not in producing it ~ which is common with "acute intermittent porphyria". Both conditions will definitely give you photoreactive urine ~ but "purple urine" is pretty much confined to "acute intermittent porphyria".
Of note, it can come out with a normal color, but sit it out in full sunlight and it'll change color. George III's courtiers noticed the color change because most palaces in those days depended extensively on natural lighting during daylight hours and, as it turns out, kings did all sorts of things in front of courtiers back then.
I have a lab appointment for 12-31-2009 to check that...thanks to you.
I never treated a case, so I’m glad you answered it. I learned something...
If I happen to be eating alot of meat and/or fish, then I usually lay off the supplement.
I never read the original article but was told that it applied to ALL Americans, one hundred percent of all those Americans age 50 and up.
Thank you for the fascinating, informative posting about porphryia.
Y O U W R O T E :
There's porphyria and there's acute intermittent porphyria. Within the classification of "intermittent" there's a problem frequently referred to as "Scandinavian acute intermittent porphyria".
Basically there are at least 86 different genes that control or set the controls for liver function. Most of them do a decent job. Some of them do a job where the intention seems to be to survive a serious starvation decade during an ice-age. Others appear to be oriented toward fending off malaria/yellowfever/etc. bearing mosquitos in tropical paradises. Whatever kind of liver function gene you have (out of that 86) none of them are "perfect" ~ but one of them is most common, and if you have anything called porphyria, you ain't got that one!
Now, for Scanderhoovians, there are three different alleles to worry about when it cames to Scandinavian acute intermittent porphyria. The Swedish/Finnish medical establishments believe there are about 500 people with the worst one, and most of the ones they know of live in Scandinavia somewhere. All of them are Sa'ami, or known to be of Sa'ami ancestry.
Since there are probably upwards of 9 million similar people in the United States, there are likely about 45,000 here who have at least one copy of that allele.
This one is, luckly, an autosomal recessive, so you need two to get the full effect WHICH CAN INCLUDE a vast host of symptoms including photoreactive urine (turns purple in full sunlight), and, at times, something akin to psychotic episodes. Remember, you get an allele from each of your parents. If you get one copy of this and one copy of the normal allele, you are probably OK. The Swedes have a genetic test for this BTW.
There are two other alleles that fall into this category. They are far more common, and the symptoms are much less disastrous. Again, both are autosomal recessives. One "symptom" is Bradycardia ~ or intermittent atrial fibrillation. This can be treated on an outpatient basis with a bit of arthoscopic surgery inside your heart.
This gene could save your life if you were prone to falling into Arctic waters on a regular basis since it'll fire up your heart to keep beating until your body temperature drops enough to turn off your brain. Your companions could then haul you to a fire and warm you up until your heart started beating again ~ BTW, this may be behind the success of some people to swim in Arctic waters for lengthy periods, or to simply survive for up to 4 hours (while everybody else dies in about 20 minutes).
"Hey" you may be saying "I thought it was all about the liver".
Obviously it's not. Genes frequently serve a multiplicity of purposes.
Another one of the side effects of all three of these genes is that the liver appears to waste a lot of time producing TWO KINDS OF HEME rather than just one. You urinate out the heme that's wrong-handed, and keep that which is correctly-handed, and build blood cells with it. The wrong-handed heme is excreted through the kidneys.
I am personally aware that the problem is SEASONAL, and usually starts with the first big jump in red-light in March, and ends in Oct/Nov. That, BTW, matches up with successful hunting seasons ~ the basic Sa'ami diet of fish, reindeer and lingonberries (and little else) results in exceedingly high levels of iron intake. If there's a toxic limit for iron in the human body traditional Sa'ami are definitely riding it like a surfboard.
Here's how it goes when the weather gets cold and you have these genes. First of all, you probably don't dress as heavily as the average guy. Or, if you have on a parka, you may unzip it and toss back the hood simply to cool down. Along with that atrial fibrillation risk, you also have the benefit of a square shaped heart. That gives you far larger than average sized atrial chambers.
You find it's cold, and your heart starts beating a little bit harder (using those atrial chambers to push blood through the lungs at a high rate), and you don't have to breath as heard, and you conserve body heat so it can be sent out to your peripheral muscles in your arms and legs so you can work in the cold. In the good old days Sa'ami worked outdoors for months at a time.
For the most part I have a hard time keeping my heart beat up to 120 beats per minute engaged in the most energetic of activities. It'll go up to that, the atrial chambers kick in, and I'll be down to 50 or less in a few seconds.
That's the sort of thing that'll wake your cardiologist up ~ and you ~ but you'll be able to survive doing heavy labor in Arctic cold.
Then there's the Vitamin B12 thing. This is the one old Aunt Sallie used to get a shot of each month so she didn't go crazy. Well, these days its a sublingual lozenge.
With the Sa'ami this all stems back to the reindeer. They eat lichens that live on bare rock. They produce all sorts of chemicals, and provide your body with high levels of selenium (and if you have a selenium deficiency that can cause you cardiovascular problems). One of the chemicals is a form of Vitamin B12 the human body can't use ~ but the liver takes it and turns it into a kind you can use. Being the mighty reindeer hunter that you are, you are consuming incredible amounts of B12 all the time because reindeer have more of it than other animals ~
The downside is that this has allowed your system to void surplus Vitamin B12 in some as yet unknown manner. Most other people who need B12 supplements simply have a condition in their intestines that restricts their absorption of B12. You can have that too, but you also have definite "leakage" somewhere.
If you believe you have Sa'ami ancestry you should probably get tested for Vitamin B12 deficiency! The pills have a pleasant taste these days, and you don't have to go in for weekly or monthly shots. They are sold OVERTHECOUNTER so no prescription is needed.
For those Sa'ami with light skin, you have a real risk of skin cancer. For those with some degree of ability to tan, you also have a real risk of skin cancer. Reading some new materials the other night I got the idea that Sa'ami should probably wear sunglasses during daylight hours ~
Anyway, I'm not an expert on this stuff. On the other hand I know a lot about it ~ and another "Sa'ami disease" called Celiac or wheat intolerance. No, not everybody has it, but one paper on Sa'ami diets indicated they simply never eat wheat or wheat byproducts.
That'll happen in families were as few as one member has Celiac. Worth reading up on that one if you are Sa'ami or have wheat intolerance. Those little tags they put up in stores these days announcing "gluten free" are lifesavers.
Sometimes I feel almost normal buying something besides meat, cheese and rice or corn products (my basic menu, but I have several jars of lingonberry preserves on hand "just in case" there's something in there I need.).
I think the notice on "everybody" was about some other B vitamin, and probably vitamin D as well.
I'll watch for it.
Anyway, it's ubiquitous nowadays to see short paragraphs such as this:
>>> "....If you're over age 50, consume vitamin B-12 in its crystalline form, which is found in fortified foods or supplements...." <<<<
Remember... this is ALL Americans, no matter what their health conditions or genetics, no matter what their Vitamin B12 status is.
I never buy the cyanocobalamin; I only purchase the methyl versions of B-12, usually just the methylcobalamin.
I'm frankly quite unsure what year the guidelines changed and that they had press releases and consequently many articles in the press about it. Perhaps it's archived away somewhere in Nexus Lexus but I'm not a subscriber.
BTW, mtDNA haplogroups 'V' and 'U' are dominant amoungst the Sa'ami people. My mother is 'V' and my dad's mother is 'U5a'...so.
I have yDNA R1b1b2 which indicates that I'm a Dane who wound up in Ireland...long ago, maybe as a Viking? (One of my dad's sisters had red hair)
Mayo is awesome. Hope your relative can get this under control.
Fantastic!!! THANKS!
B12 as a supplement doesn't work if you take Glucophage ~ so you'll need sublingual B12 ~ just thought everybody reading this thread would like to know that.
A friend of mine, who shares a huge number of ancestors going way back, had surgery in the lower bowel area ~ and developed Celiac-like symptoms ~ he's had the skin cancer, the erratic heart beat, Vikings hand, and a bunch of other stuff ~ but this one is new and he is not prone to change his diet (no soy sauce, no beer, no bread, no.....) so he's not getting a test.
You don't have to live your life like that. The Sa'ami got along fine without beer and bread for thousands of years ~ so can you!
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