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Curious Clips: Excerpts from the Curious Report

Wednesday, September 13

 

THE ROYAL DISEASE

On March 27, 1884, one of Queen Victoria's sons fell down some stairs at a yacht club in southern France. The accident was minor; Prince Leopold suffered nothing more than a few bumps and bruises, but the 31-year-old Duke of Albany had hemophilia, a disorder that prevents the blood from clotting. His injuries triggered massive internal bleeding. Within 24 hours, he was dead.
 
Though Victoria never suffered from hemophilia herself, she carried a genetic mutation that causes the condition and passed it on to several of her offspring. In Queen Victoria's case, both of her parents appear to have had functioning blood-clotting genes. However, due to a copying error during reproduction one of hers was dysfunctional. Such spontaneous mutations are uncommon, but far from unheard of - they pop up in about one person out of 50,000. Victoria passed her deficient blood-clotting gene to only one of her sons - the unfortunate Leopold. She also passed it to two of her daughters, Beatrice and Alice.
 
The Next Generations
Leopold, Beatrice and Alice had 12 offspring in all. Leopold's boys were unaffected because they got their X chromosomes - and thus their blood-clotting genes - from their mother. But two of Beatrice's sons, and one of Alice's, inherited the faulty version of the X. Alice's son Frederick died as a young boy after smashing through a window. Beatrice's son Leopold bled to death at the age of 32 during a knee operation. Maurice, her other hemophiliac son, escaped a similar fate, only because he was killed instantly by an exploding shell during World War I.
 
On the female side, Leopold and his sisters had four daughters who inherited the faulty X chromosome and spread it widely through the royal houses of Europe. During the early 20th century, princes of Spain, Prussia, Russia and Great Britain suffered from what became known as "The Royal Disease."
 
What about the royals of today? Oddly enough, Victoria's deficient blood-clotting gene never made it beyond the fourth generation. By sheer luck, none of Victoria's great-great granddaughters in Spain or Britain inherited it. In Prussia she had only great-great grandsons. And none of her Russian great-great granddaughters ever had any children; all four were murdered during the Bolshevik revolution.
 
What type of hemophilia was “The Royal Disease”?
Because the last known descendant of Queen Victoria with hemophila died in the 1940s, the exact type of hemophilia found in this family remained unknown until 2009. Using genetic analysis of the remains of the assassinated Romanov dynasty, and specifically Tsarevich Alexei, scientists were able to determine that the “Royal Disease” is actually hemophilia B. Specifically, they found a single-nucleotide change in the gene for clotting Factor IX that causes incorrect RNA splicing and produces a truncated, nonfunctional protein.
 

 

Curious Clips: Excerpts from the Curious Report

Wednesday, September 6

WHAT IS HEMOPHILIA A?

Hemophilia A, also called factor VIII (FVIII) deficiency or classic hemophilia, is a genetic disorder caused by missing or defective factor VIII, a clotting protein. Although it is passed down from parents to children, about 1/3 of cases are caused by a spontaneous mutation, a change in a gene.

According to the US Centers for Disease Control and Prevention, hemophilia occurs in approximately 1 in 5,000 live births. There are about 20,000 people with hemophilia in the US. All races and ethnic groups are affected. Hemophilia A is four times as common as hemophilia B while more than half of patients with hemophilia A have the severe form of hemophilia.
 
Genetics
The X and Y chromosomes are called sex chromosomes. The gene for hemophilia is carried on the X chromosome. Hemophilia is inherited in an X-linked recessive manner. Females inherit two X chromosomes, one from their mother and one from their father (XX). Males inherit an X chromosome from their mother and a Y chromosome from their father (XY). That means if a son inherits an X chromosome carrying hemophilia from his mother, he will have hemophilia. It also means that fathers cannot pass hemophilia on to their sons.
 
But because daughters have two X chromosomes, even if they inherit the hemophilia gene from their mother, most likely they will inherit a healthy X chromosome from their father and not have hemophilia. A daughter who inherits an X chromosome that contains the gene for hemophilia is called a carrier. She can pass the gene on to her children. Hemophilia can occur in daughters, but is rare.
 
For a female carrier, there are four possible outcomes for each pregnancy:
1. A girl who is not a carrier
2. A girl who is a carrier
3. A boy without hemophilia
4. A boy with hemophilia
 
Symptoms
People with hemophilia A often bleed longer than other people. Bleeds can occur internally, into joints and muscles, or externally, from minor cuts, dental procedures or trauma. How frequently a person bleeds and the severity of those bleeds depends on how much FVIII is in the plasma, the straw-colored fluid portion of blood.
 
Normal plasma levels of FVIII range from 50% to 150%. Levels below 50%, or half of what is needed to form a clot, determine a person’s symptoms.
  • Mild hemophilia A: 6% up to 49% of FVIII in the blood. People with mild hemophilia A generally experience bleeding only after serious injury, trauma or surgery. In many cases, mild hemophilia is not diagnosed until an injury, surgery or tooth extraction results in prolonged bleeding. The first episode may not occur until adulthood. Women with mild hemophilia often experience menorrhagia, heavy menstrual periods, and can hemorrhage after childbirth.
  • Moderate hemophilia A: 1% up to 5% of FVIII in the blood. People with moderate hemophilia A tend to have bleeding episodes after injuries. Bleeds that occur without obvious cause are called spontaneous bleeding episodes.
  • Severe hemophilia A: <1% of FVIII in the blood. People with severe hemophilia A experience bleeding following an injury and may have frequent spontaneous bleeding episodes, often into their joints and muscles.

 


 

Curious Clips: Excerpts from the Curious Report

Friday, September 1

WE ARE EXCITED ABOUT...

Highlighting a playwright whose writing roots are grounded in San Diego. Karen Hartman, a nationally recognized playwright who had three distinct world premiere plays produced in the 2016-17 season, grew up in San Diego and found her love of writing plays through our local Playwright’s Project. She went on to graduate from Yale University and now works as a Senior Artist-in-Residence at the University of Washington in Seattle.

We are proud to welcome Karen back home and re-introduce her to many in our theatre community who have yet to experience her craftsmanship. Roz and Ray, still a relatively new piece of theatre, is on its third production (the World Premiere co-production was seen last season at Seattle Repertory Theatre and Victory Gardens in Chicago). Plus, the play carries the distinction of being her first professional production in San Diego.
 
As a regional theatre, we are also dedicated to showcasing stories about the place we call home. Roz and Ray does just that. It is set in San Diego in the 1970s. Ray, a newly single parent of twin hemophiliac boys, has only one goal: keep his sons alive. His days are filled with endless trips to the hospital, rigorous testing, and frequent blood transfusions. This all changes when Ray meets Roz, an optimistic and caring doctor with a miracle drug. Roz appears to be Ray's savior until the miracle turns into a nightmare. In part, the story is based on Karen’s father, who was a Hematologist/oncologist at Rady Children’s Hospital and worked with hemophiliac patients during the AIDS crisis. It comes from a very intimate exploration of a little known piece of medical history.
 
As noted in Forbes, Karen’s play, “brilliantly captures the confusion, and the moral ambiguity about life on the edge of biomedicine.” But Karen also went straight to the heart of the subject, humanizing a medically-infused storyline through two complex characters: Roz and Ray. “I think of it as a very complicated unconventional love story in the midst of a very brutal and difficult time,” Hartman said in an interview with the Alley Theatre. She went on to say, “I had the idea for about ten years before I had the guts to write it. It's a medical morality story that's never been told in this exact way. I feel a lot of sympathy on both sides. These two characters are really good and very imperfect people and they go through a lot together.”
 
We are also excited to have compiled the ideal team for this production that reminds us of the powerhouse talent we have right here in San Diego. Veteran San Diego director Delicia Turner Sonnenberg leads Carla Harting (last seen on the REP stage in Outside Mullingar) and Steven Lone (last seen on our stage in Zoot Suit), in a truly intimate battle of the heart. Both, by the way are UCSD grads!
 
The team is also supported by an excellent combo of designers including: John Iacovelli (scenic,) who crafted the swanky set for our production of Disgraced last season, Shelly Williams (costume,) who is on staff at The Old Globe, Sherrice Mojgani, who is a local lighting/projections favorite, plus the REP’s very own Matt Lescault-Wood (sound.) Come prepared for a little bit of visual poetry to carry you through this emotional piece.
 
Our Associate Artistic Director Todd Salovey produced this production. He, and the rest of the Artistic Team, are thrilled to be able to bring this locally-based story, written by a San Diego native, starring top quality actors from our theater community with a director and designers who have become a beloved part of our REP family! This production is truly a regional theater extravaganza and hits the mark on what San Diego REP promises in their mission.
 
We now look forward to sitting with you as the lights dim and the storytelling starts. See you in the theatre.

 

 

 
 
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