The Czarevitch
As we now know the Czarevitch Alexis suffered from hemophilia b. Does anyone
know how the boy would be treated for it in the world of today?
Thank You
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I remember many years ago when I was a kid (early 1970s), there was a commercial that came up quite a bit, where it showed a kid who was a hemophiliac and spoke about a clotting factor that his family couldn't afford. There was another commercial which showed a kid from a rich family who could afford the clotting factor, so he was obviously better off.
http://en.wikipedia.org/wiki/Haemophilia
Like most aspects of the disorder, life expectancy varies with severity and adequate treatment. People with severe haemophilia who don't receive adequate, modern treatment have greatly shortened lifespans and often do not reach maturity. Prior to the 1960s when effective treatment became available, average life expectancy was only 11 years.[5] By the 1980s the life span of the average haemophiliac receiving appropriate treatment was 50–60 years.[5] Today with appropriate treatment, males with haemophilia typically have a near normal quality of life with an average lifespan approximately 10 years shorter than an unaffected male.[7]
...
Though there is no cure for haemophilia, it can be controlled with regular infusions of the deficient clotting factor, i.e. factor VIII in haemophilia A or factor IX in haemophilia B. Factor replacement can be either isolated from human blood serum, recombinant, or a combination of the two. Some haemophiliacs develop antibodies (inhibitors) against the replacement factors given to them, so the amount of the factor has to be increased or non-human replacement products must be given, such as porcine factor VIII.
If a patient becomes refractory to replacement coagulation factor as a result of circulating inhibitors, this may be partially overcome with recombinant human factor VII (NovoSeven), which is registered for this indication in many countries.
In early 2008, the US Food and Drug Administration (FDA) approved Xyntha (Wyeth) anti-haemophilic factor, genetically engineered from the genes of Chinese hamster ovary cells. Since 1993 (Dr. Mary Nugent) recombinant factor products (which are typically cultured in Chinese hamster ovary (CHO) tissue culture cells and involve little, if any human plasma products) have been available and have been widely used in wealthier western countries. While recombinant clotting factor products offer higher purity and safety, they are, like concentrate, extremely expensive, and not generally available in the developing world. In many cases, factor products of any sort are difficult to obtain in developing countries.
So, if it was available, I'm sure the Romanovs would have been able to afford it.
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I knew two hemophiliacs in college in the late 1970s, my roommate and his brother. They managed their illness quite well with clotting factor, a clear liquid that came in small bottles (didn't need refrigeration) that they would inject into the area where they were bleeding or having pain. Of course I don't know how expensive it was, but I know they were both at school on scholarships and worked part time. They got the clotting factor through a local hospital that is world renowned for work on blood disorders, so there may have been some kind of research project they were part of. I don't know anything more specific.
Unfortunately the clotting factor came from donated blood, and they both contracted AIDS in the late 70s, before there were reliable tests for it, and died.
That is so tragic!; please accept my condolences.
God is subtle, but He is not malicious. (Albert Einstein)
Thank you for posting this Stevicus-2. I am an active member in the hemophilia community. The Romanov's are my distant cousins. Or should I say my ancestors. What people do not know is that women also get symptoms of hemophilia. They are called symptomatic carriers.
Laura
people do know that. women can only ever be carriers of it, men are the ones affected. for obvious reasons, women can't be haemophiliacs
for obvious reasons, women can't be haemophiliacs
That is not true. Woman can be hemophiliacs, but it is rare for this to occur. The hemophilia gene is carried on the X chromosome, which is passed along the female line. Women have two X chromosomes, men have an X and a Y. A female carrier of hemophilia, like Alexandra, has one normal X chromosome and one mutated gene on the X chromosome for hemophilia; a male who inherits the hemophilia X chromosome from his mother will be affected; a daughter of a carrier parent has a 50/50 chance of inheriting the hemophilia gene rather than the normal gene, as she gets one X chromosome from her mother, whose genetic composition is two X chromosomes, one being normal.
So, in order to be an active hemophiliac, not merely a carrier, a female has to inherit an affected X chromosome from EACH parent. If her father is an affected hemophiliac, like Prince Leopold (Queen Victoria's son) he will pass on his affected X chromosome to EVERY daughter, but his sons, if any, will be unaffected and will not be carriers, since they do not get an X chromosome from their father. He passes on only the Y chromosome to his sons. So, if the daughter of a hemophiliac father ALSO inherits a hemophilia gene on the X chromosome from a carrier mother, she will have two hemophilia X chromosomes, and thus will be affected.
This is a very rare event, but it is possible. Hemophilia will never be eliminated entirely (at least, with our current knowledge and technology) because some 40% of cases are due to spontaneous mutations and thus no family history could warn the parents-to-be.
The Center for Disease Control has a fact sheet with the inheritance mechanism clearly explained, and the note that women can also be affected when they get the hemophila X chromosome from both parents:
http://www.cdc.gov/ncbddd/hemophilia/facts.html share