Rumor has it part of my family tree goes back to Russian royalty.....

 

Hi Jeff,

You might want to peruse this list to see if you recognize anyone. You never know.

Most of my ancestors weren't even close to royalty. They were, shall we say, a bit rough around the edges. My father's family came to America one step ahead of the authorities.

Mike

http://www.btinternet.com/~allan_ray...yal_Family.htm

 

Thanks, that's pretty neat.. I've meant to do some research on my Mother's side of the family. The Russian Royalty stuff started with the rarity of my genetic bleeding disorder (Hemophilia B). It is supposed to have originated in Russia's Royal Family. Also, the Hungarian part of that side of the family supposedly fled to Hungary from Russia??? That's why I said it was a rumor....

 

I'm just glad to know that none of my ancestors were ever found to be swinging by their arms nor from their necks from any branches of our family tree.

 

So Jeff,

If you cut yourself, what do you have to do to stop the bleeding? Can you find yourself in danger of bleeding to death? I went to high school with a guy who had hemophilia, and he had to be extremely careful all the time. Couldn't play sports, etc. Of course, that was 40 years ago and I'm sure that medical science has come a long way since then....

What about passing on the genetic trait to your children? If I remember correctly, this is primarily a male issue, so sons could be at risk but not daughters - right or wrong?

 

Here is the quick lesson. I have Hemophilia B (very rare) that is similar to Hemophilia A. A is severe but most patients live normal lives with daily infusions of blood products. B is only a risk if / when I have a severe trauma or surgery. The biggest danger to any Hemophilia patient is the risk of AIDS. This was a major issue in the 80's when quite a few of the Hemophilia patients at my center in Philly died. Now we have a synthesized product that eliminates the chance of getting AIDS. It is WAY expensive. The last treatment I had about 5 years ago cost over $150,000.00. No, that isn't a typo. I still have the bill. Thank God for insurance. Now when your done cleaning the coffee off your screen I'll explain how it is passed.

It is passed through the females of the family and is only contracted by the male children. So, my sister has the potential to pass it to her sons, and her daughter carries it. Here is where it is a pain to contain. It doesn't always show up. My sister has two sons, both don't have it. My Aunt (Mom's sister) had a son and daughter. The son didn't have it. Her daughter had two boys and one has it and one doesn't. So, once in the family line it never goes away but also doesn't always show up. My kids are of no risk since I don't pass it on. Since it is always a marker in the female line hence my comment about Russian royalty.....

I tried to make it short....

 

Thanks for the quick lesson. How long do these treatments remain in effect, and what do they improve for you? Again, what actions must you take now if you cut yourself shaving or scrape your knuckles working on a car? You say that severe trauma or surgery is risky, but what about everyday occurences like these? I assume minor cuts and scrapes will clot up eventually, but maybe you must take special precautions. That's what I'm trying to understand here....

 

The basic mechanics of it: Your blood platlets are made up of 16 different clotting factors. Think of them as the ingredients of a beef stew. They all are needed but some play a bigger role than others. Hemophilia A patents are deficient in factor 8 while Hemophilia B patents are deficient in factor 9. Think of Hemophilia A as the beef in the stew and Hemophilia B as the potatoes. There is no breakdown of the treatments. The factor will eventually be used by the body. Once it is used up, it is gone. My body doesn't reproduce enough to replace what it uses. As far as injury goes, I'm fine. Some cuts will bleed a little longer, but eventually all the injuries will heal... Actually some of my bruises have been pretty wild to watch. They can get real big and as the blood settles I will show bruising far from the injury site..Like when I hurt my shoulder and elbow and parts of my forearm and wrist turned purple...That is where the blood settled...

Does that help?

Hey this just in from Wiki (Checking my own facts since it's been a while since I've given a dissertation on Hemophilia. )

British and Russian Royal Families
A study published in 2009 identified the blood disease affecting both the Russian and British Royal Families as haemophilia B on the basis of genetic markers.[3][4]

Also don't know if I really do have "Royal" blood??? Here is an interesting article...
http://news.sciencemag.org/scienceno.../10/08-02.html


I have also learned that I do pass the "carrier" gene to a daughter....Oh boy... My previous post about the marker being passed from female to female were incorrect. It is passed from father to daughter..Oh boy, that is going to be a hard conversation with Olivia.

 

I have a question regarding your explanation...

Is the AIDS risk due to use of someone else's blood components as part of the therapy?

Or does the missing clotting component somehow increase the potential for infection with the HIV virus (or it's progression to full blown AIDS). HIV testing has come along way since the 80's especially with the new DNA based PCR tests, that should make the use of synthetic products not really necessary.


George

 

Over here (UK), it was that the blood was (for a time) not screened (despite campaigns for it to be screened) so anyone being given blood could also be given HIV

Most blood was not contaminated..... but it was still a sort of Russian roulette

I also was at school with a haemophiliac - but didn't know there was more than one type.

 

Ah, yes....Lemme explain...

The AIDS infections were from tainted products. Yes, screening is MUCH improved but no one will guarantee a 100% protection when taking a straight blood product. So,would you take the chance if they said "well we're 98% sure it's safe"? The synthesized (not synthetic, ha gotcha, reread the post...Na na na) product is strictly the missing protein (factor) from the plasma and is 100% safe from any blood borne pathogens including AIDS and Hepatitis.

Wow, I never thought anyone would take that much interest..It's mainly a PIA that I have to live with and ever since the advent of AIDS no life insurance co. will touch me...

 

Wow... I understand now. However you're chances of contracting HIV from a medical transmission in the western world from are 1 in 2.5 million at this point, at least from the most recent World Health Organization Report I read. (I'm an electrical engineer but I work as the IT director for a drug company, so a lot of "interesting" stuff comes across my desk).

Your chances of contracting HIV just being well a human and going about your life are much greater. (albeit not much higher 56k average infections per year, spread across 320 million people in the US) Personally I think it's a cop-out from the insurance co and an excuse not to cover someone who they think may prove to be of a higher risk.

Granted I understand the "better safe than sorry" position that both you and the medical facilities take.

George