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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.).