Hemophilia B

What a doll!

Our eminent researcher of all things hemophilia, Richard Atwood of North Carolina, has found a book with “paper” dolls related to hemophilia! I certainly had paper dolls growing up, and apparently, we still have them for kids these days.

Nicholas and Alexandra Paper Dolls, published in 1998, is an oversized book (9”x12”) on the family of the last Romanov Tsar, Nicholas II, whose son Alexei had hemophilia B. The book includes 11 dolls on 16 plates printed on lightweight cardboard. There are two pages of text with notes and instructions to provide historical insights and fashion commentary. Alexandra has two dolls, accompanied by six dresses, including her 1894 wedding dress and 1896 coronation dress, plus ball gowns. Nicholas has two dolls, accompanied by five uniforms. Alexei’s older sisters, Olga, Tatiana, Marie, and Anastasia all wear the same casual dresses, that may be covered by two sets of identical dresses. Alexei is dressed in a sailor suit that may be covered with a uniform. Other dolls are the mad monk Gregory Rasputin in a monk’s garb and Clementy Nagorny in a sailor suit. The book cover illustration could also be used as dolls for Nicholas, Alexandra and Alexei.

The life of Alexei is briefly summarized. Alexei was born in August of 1904. From his mother, he inherited hemophilia B that was soon evident to the family, who did not publicly reveal it. Hemophilia was a deadly disease then, with no known cure. The bleeding bouts caused pain. Gregory Rasputin is described as a healer who used hypnosis to relieve Alexei’s suffering. Rasputin is also described as a lecher, a drunkard, and a con man. Clementy Nagorny was one of two sailors who acted as Alexei’s nanny.

Alexei is correctly shown with a bent left leg, though the metal brace he wore for most of 1913 is hidden. Something not revealed in the text is that Alexei’s accident at Spalo in 1912 caused femoral neuropathy (or paralysis of femoral nerve) due of an iliacus hematoma (or an iliopsoas muscle bleed). This was the medical reason for his hip flexion and bent leg. The metal brace, not Rasputin, straightened Alexei’s left leg. The instructions printed in the book do not mention the need for a sharp cutting tool such as an X-Acto knife in order to remove the dolls and clothes from the book pages.

The author, Tom Tierney, who is an artist, has signed each of the 11 dolls. Tierny is known for his series of paper dolls for a variety of historical figures, including Queen Elizabeth II, Michelle Obama, Abraham Lincoln, Shakespeare characters, and more.

And, you can still buy this on Amazon.com!

From Mineola, NY: Dover Publications. 32 pages.

New Episodes of the Infusion Squad Take Kids with Hemophilia B on a Mission into the Bloodstream

This is a paid public announcement from Sanofi and does not constitute an endorsement of products or services. When you click on the links in this blog entry, you will be directed to a Sanofi website. LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

Two new episodes of the Infusion Squad are now available from Sanofi to take kids with Hemophilia B on an audio adventure through the bloodstream.

Anyone who deals with children knows that getting them to sit still for more than a few minutes can be mission impossible. This can be especially challenging for children with hemophilia B who may receive regular infusions of Factor IX therapy that helps them manage their hemophilia. 

For entertainment and education during the infusion process for children and their caregivers, Sanofi launched the audio adventure series, Infusion Squad, available as an Amazon Alexa voice skill for patients in the U.S. The now ten-episode series is designed for children aged 12 and younger but is fun for any age.

Part educational and part entertaining, each episode takes a team of “Infusionauts” and the children on a mission into the bloodstream. Along the way, children learn more about infusion and the role that factor IX plays in hemophilia B. 

The series was created based on patient research and focus groups that found it can be challenging for children to stay still during the infusion process. They also expressed the need for educational materials to help families learn about hemophilia B and infusing. 

Available in English, the Infusion Squad skill can be enabled on the Alexa app, Alexa Skills store, or on any Alexa-enabled device. Just ask Alexa to “enable Infusion Squad” and you’ll be dropped right into numerous episodes of hands-free adventure.

To learn more about the Infusion Squad or a treatment option for Hemophilia B, visit https://bit.ly/30mc6fE . You can also contact your local Sanofi Community Relations and Education Manager here.

© 2022 Genzyme Corporation. All rights reserved. Sanofi is a registered trademark of Sanofi or an affiliate. Amazon, Alexa and all related logos are trademarks of Amazon.com, Inc. or its affiliates.


Merry Christmas! (disease)

I wish everyone a Merry Christmas and joyous new year.

Christmas reminds me that factor IX deficiency is also known as “Christmas disease.” Did you know that the name has nothing to do with the holiday?

The name comes from the first person properly diagnosed with factor IX deficiency, a Mr. Stephen Christmas, born a British citizen in 1947, who emigrated with his family to Canada. He was diagnosed at age 2 with hemophilia (no type yet). On a return visit to England in 1952 Stephen was diagnosed by the Oxford Haemophilia Centre as not having a normal case of hemophilia. Research led to a new classification, called factor IX due to the low levels or absence of factor IX protein in the blood. This eventually led to many others being properly diagnosed. Although Stephen later contracted HIV, he became an advocate for the screening of donated blood to increase the safety of transfusions and infusions.

And he went down in history: the new disorder was named after him!

Interesting Book I Just Read
I, Steve: Steve Jobs in His Own Words
George Beahm

I was too intimidated at first to read the new Steve Jobs book by Walter Issacson, so I settled for a short and sweet book. Well, it’s short! It is a collection of quotations by Steve Jobs, about business and life. It’s nice in that it’s a quick read, and you can go back and browse through it. Many of the quotations seem to be space fillers, and trite, shedding no light on Jobs or on anything! But some of it is good. I suspect you could get a lot of this on line, and save a few bucks.

Also, I finally finished Steve Jobs, which I will write about next week, and it makes I, Steve present Jobs as a savvy and wise man, sugar-coated all the way. To know the real Steve Jobs, read the Issacson book. For snippets, this is fine. Two stars/five.

The Royal Disease Defined

You probably already know that hemophilia is referred to as the “royal disease.” It’s not a royal disease, of course, but it was made famous by the royal families of Europe, some members of which had the blood disorder. Britain’s Queen Victoria is the world’s most famous carrier: she had nine children, but only son Leopold had the disorder.

Up till now, no one knew which type of hemophilia the royal line carried: A or B?

Read Nicolas and Alexandra, by Robert K. Massie, if you want a fascinating look at history that reads like a love story. This was also made into a spectacular movie. And for children, I offer my own book Alexis: The Prince Who Had Hemophilia, free of charge through my website. If you have hemophilia in your family, this is a story you must know!

The results are in; the DNA results, that is. Britain’s Queen Victoria’s family had a severe form of hemophilia B. Our own University of Massachusetts’s Dr. Evgeny Rogaev has conducted DNA tests on the remains of the Romanovs, the queen’s Russian relatives. Victoria’s granddaughter Alexis married Nicholas II, the last Russian tsar. Rogaev examined the remains of Prince Alexei, himself, who had hemophilia, and who was killed with his entire family on Lenin’s orders.

This is one of history’s most fascinating stories: how hemophilia played a part in the overthrow of the Russian dynasty and perhaps led to the Communist take-over. Communism helped to define much of world history following World War I.

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