The Most Famous Hemophilia Carrier

I’m preparing for a visit to Russia next
week, a country I have not been to since 1999. So I’ve been reading two
excellent books to prepare: Peter the Great and Nicolas and Alexandra, both by
Pulitzer-prize winning author Robert K. Massie.

These books, and last week’s post on
hemophilia myths, got me thinking: Do you know who the most famous carrier of
hemophilia was? Hint: It’s why hemophilia is dubbed “The Royal Disease.”
Queen
Victoria of England, one of England’s longest reigning monarch. Her 63-year reign became known as the Victorian era, and was the longest reign until
September 9, 2015, when Queen Elizabeth II surpassed her. Her era saw British
power at its zenith across the globe; Victoria believed that the British Empire
existed to civilize people in less developed countries and to protect them from
their own rulers and the aggression of neighboring rulers. Others saw the
purely commercial aspect of this world domination of lands and trade routes.
Victoria took the throne at age 18, and later married
her first cousin, Prince Albert of Saxe-Coburg and Gotha, in 1840. Over the
next seventeen years, she and Albert had nine children: Albert
Edward, (b. 1841), Alice (b. 1843), Alfred (b. 1844), Helena (b. 1846), Louise (b. 1848), Arthur (b. 1850), Leopold (b. 1853) and Beatrice (b. 1857).
The last child, Beatrice, was delivered under the care of Dr. John Snow, who
later became the founder of modern public health by discovering the
transmission mechanism of cholera (infected water). Snow used anesthesia on
Victoria, thus giving her pain-free childbirth for the first time! While Snow
did not invent anesthesia, he created a more convenient and safer way to administer
it.
When their nine children married into royal and noble families throughout Europe (for mostly
political reasons), Victoria was called “the grandmother of Europe,”
and indeed was called “Granny” by all her grandchildren and their spouses. 
Hemophilia was not known to exist in the
royal family before, but Victoria carried the gene for hemophilia B. Only
Leopold had hemophilia; two daughters, Beatrice and Alice, were carriers. They
later transmitted hemophilia to the Spanish and Russian royal families. 
Leopold grew up to be a tall, intelligent,
affectionate yet stubborn prince, whose willfulness often led to injuries and
bleeds, according to author Massie. The Queen was unusually attached to her
son, and worried over him incessantly. Victoria reported in one letter that
Leopold had been at death’s door four or five times. Eventually Victoria tried
to keep him confined to the upper floors of Buckingham Palace for his own
protection, even as a man! But he managed to get away to Paris for two weeks.
Eventually at age 29, he fell in love with a German princess, Helen of Waldeck.
They had a daughter. When Helen was pregnant a second time, Leopold fell,
suffered a brain hemorrhage and died at age 31.
 There was no treatment for hemophilia then, of course. Victoria was informed by telegram that her youngest son had died in Cannes. He was “the dearest of my dear
sons,” she lamented.
According to one of her
biographers, Victoria wrote an average of 2,500 words a day during her adult
life, and kept a detailed journal, which eventually encompassed 122 volumes. From this we
learned a bit about hemophilia in those days.  The
Queen didn’t know what type of hemophilia her son had or if there even were
types; only much later, through genetic testing, was the family found to have
hemophilia B. While the mechanisms weren’t entirely understood, the royal
family knew that hemophilia could be passed down from generation to generation.
And it was; when it hit the Russian royal family, it would change the world.

For more information, read Robert K. Massie’s excellent books, or view “Nicolas and Alexandra,” a major motion picture and excellent portrayal of the last years of the Russian monarchy. Available on Amazon.com.  Robert K. Massie also has an adult son, Robert K. Massie, Jr., who was later cured of his hemophilia through a liver transplant. His life is exquisitely portrayed in the book Journey.

Putting One of Our Own in the Senate


There was a welcome article when I picked up the Boston Globe this morning: “REMEMBER BOB Massie? The 1994 candidate for lieutenant governor doesn’t think so, and he’d like to reintroduce himself. “I’ve been away a long time,’’ he said in an interview.”

Of course we remember him. Son of Pulitzer Prize winning author Robert K. Massie, and author Suzanne Massie, who served as one of Ronald Regan’s advisors on Russia. They are most famous for the book Nicolas and Alexandra, the last Tsar and Tsaress of Russia who had a son with hemophilia. High powered parents, and an extraordinary childhood, all chronicled in the parents’ deeply stirring book Journey, arguably the first book ever on hemophilia. Many of us know Bob, and many of us just saw him at NHF in New Orleans. Yes, he has been away, with good reason. He no longer has hemophilia, thanks to a liver transplant in July 2009.

Bob has been through a lot. I saw him last January, when he donated all of his remaining factor to Project SHARE; with a new liver, he no longer has hemophilia and doesn’t need factor anymore. I visited him in his Cambridge home and got to meet one of his sons. I noticed all the photos of Bob with politicians: Bill and HIllary, Al Gore, and Ted Kennedy. His family has close political ties with many of our country’s top figures. And when we saw him in New Orleans in November, his transformation was remarkable. He looks fabulous, and obviously feels great. He is now a contender to unseat Republican Scott Brown in the Massachusetts Senatorial race in 2012.

Bob’s the first Democrat to publicly declare his candidacy. The Globe article notes: “Massie is certainly an unusual candidate. An ordained Episcopal priest with a PhD from Harvard Business School, he
is an award-winning author and social entrepreneur who also happens to be one of the longest-surviving HIV
patients on the planet.

“Is he a strong candidate? His ability to raise money and build an organization will tell…. he also knows he needs to get an early start on 2012, and is performing the necessary obeisance to political figures around the state. He formed a campaign committee, is establishing a depository account, and will have a website up shortly.”

For us, the hemophilia community, Bob could be a true godsend for health care reform. The Globe reports, “He believes
the American medical system (at least until the recent reforms) is a disgrace: tending to deny care to those who
need it most. ‘In addition to the burden of illness, people are being punished — there’s no other word for it — with
bankruptcy, misery, poverty,’ he said. ‘In my view that’s un-American.’

“…Massie has also deeply studied the health care system, its economics and history, from the inside out. At age 12 his family spent a year in France, where all his hemophilia drugs and treatments were covered ‘as a fundamental right of citizenship.’ Health care policy is not theoretical with him. Besides, Massie is no anti-business scold. He has worked with many of the world’s largest companies, including Sunoco and Ford, to develop the first standardized measures of corporate social responsibility.”

It will be interesting to see if Massachusetts, which stunned the nation when Scott Brown was elected, is ready to re-evaluate. Long considered a liberal state, and fertile ground for Democrat hopefuls, Brown overturned all the tables. Bob could represent a return to the roots of liberalism in Massachusetts. His candidacy can perhaps be a litmus test to see how Massachusetts is transforming. Was Brown a blip? We will wait and see. The idea of having someone who has suffered from hemophilia and hepatitis most of his life at Capital Hill could only be great for the hemophilia community, and perhaps for all who suffer from chronic disorders.

From “The timely return of Bob Massie”
By Renee Loth
January 16, 2011
Renee Loth’s column appears regularly in the Globe.
© Copyright 2011 Globe Newspaper Company.

A Christmas Gift of Life

Bob Massie was given a second chance at life this summer. In a previous blog I told how this famous person with hemophilia, made famous by his own parents who tell his life story of growing up with hemophilia in the 1960s and 70s in the incredible book Journey, received a liver transplant this summer in Atlanta, in a historic operation. Bob is doing much better, and looks great. I was honored to get a phone call from him, and an invitation to his home in Somerville. I visited him last week.

I hadn’t seen Bob in years, not since he decided to run for the office of Lieutenant Governor of Massachusetts, the first HIV infected person to do so. It was great to see him, and I had kept up with him periodically through his sister, Susanna, who also has a son with hemophilia. Bob wanted more than to just visit; he wanted to give back the gift of life: his remaining inventory of factor.

His gift of 120,000 IUs of factor will indeed give life to many in developing countries. We have a long list at Project SHARE, and this meant tooth extractions, surgeries and emergency stores could be provided.

The Massie’s home is lovely, one of those solid, stately colonial homes that is so firmly built it could withstand a hurricane. Somerville is a densely populated city, with little space. Houses sit in a row, closely together. My husband grew up in Somerville, where he told me his back yard basically was the city. The Massie’s house has an ample back yard, with garden and trees. Inside, framed photos are everywhere: his family, including his lovely wife Anne, and his three children, John, Sam and Katie. A photo of young Bob in a wheelchair with Muhammad Ali caught my eye. I remember the wheelchair shot (sans Ali) in the book Journey. Other photos included Bob with Hilary Clinton, Bill Clinton, Al Gore, the list goes on and on.

I got to meet John, who is a college student, a polite and friendly young man. It sounds like the Massies are all doing well; mother Suzanne, who is an expert on all things Russian and served even as advisor to president Ronald Regan, still at the age of 79 dashes off to Russia and continues her work there. Amazing, but when you read Journey, you are reminded again of her strength.

From the many people around the world this holy week of Christmas who will receive a most wonderful gift of factor, a gift of life, thank you, Bob Massie. We all wish you continued good health and happiness!

Great Book I Just Read
What Strength Remains by Tracy Kidder

I’ve been a fan of Kidder’s since reading (three times) Mountains Beyond Mountains. In this wonderful book,
Kidder tells the remarkable story of Deo, a third-year medical student, and a refugee from Burundi, who arrives in New York in 1994 penniless and suffering from post-traumatic stress disorder. He escaped one horror to face new struggles: no knowledge of English sleeping in abandoned tenements in Harlem, working for $15 a day. Occasionally Deo, who survived horrors in Burundi, is disturbed by flashes of memory of his past. Kidder accompanies Deo for 6 months, documenting his progress, recording hsi story and eventually returns to Burundi with him. When recording hsi story about the genocide in war torn Burundi and Rwanda, Deo panics. Deo describes what his people call “gusimbura,” the ability–the unwillingness– not to recall bad things, and begs Kidder not to “gusimbura” him. He wants to forget.

Kidder is one of our greatest skilled, journalist writers; the story moves effortlessly, and deeply: Deo is first befriended by a nun, then an older couple, a sociologist and his wife, an artist, who pay for him to enroll at Columbia University’s School of General Studies. He eventually gets to medical school; you will be inspired at his determination and intelligence. All seems welll. But all is not well. His return to Burundi is heartbreaking and healing; in the end, a glorious thing happens. Please read this book: it will make a wonderful holiday gift for someone you know–or yourself! Be inspired by the amazing life of Deogratis, whose name means in Latin, “Thanks to God.” Four stars.

Bobby Massie Cured


The Massie family is about as close as the US has ever come to having a royal hemophilia family. Robert Massie Sr is a Pulitzer Prize winning author, famous for his books Nicolas and Alexandra and Peter the Great. He began writing about Russian royalty while working as an editor for Newsweek, after the birth of his son Bobby, who was born in 1956 with hemophilia. Suzanne Massie is also an author, mostly on Russian culture and history, such as The Land of the Firebird (of which I own an autographed copy). They are both fabulous writers.

But it was their joint book Journey which sent shock waves to the world. For the first time, hemophilia was documented in all its detailed pathos. Both parents write in alternating chapters: he focusing on the scientific aspects mostly, she on the emotional aspects. It makes for a gut wrenching, blistering page-turner; a roller coaster of emotions at a time when there was no factor concentrate. The Massies were propelled to stardom following the release of Nicolas and Alexandra, which was later made into a movie. There were movie premiers, evenings with celebrities like Stephen Stills, Oscar de la Renta, and dinner at the White House.

I was a huge fan of Robert K. Massie by age 25, even before my own son with hemophilia was born. Call it weird, fate or whatever, but there I was, in labor September 8, 1987, reading Peter the Great while waiting for Tommy to be born. When Tommy was circumcised 12 hours later and the bleeding wouldn’t stop, I suddenly recalled my mother’s words from a few years ago, when she gave me the book as a Christmas gift: “The author writes about Russian history because his own son has hemophilia.”

Little did I know Bobby Massie lived only two miles from my house at the time, Through our treatment center nurse, I was introduced to someone whose whole family I admired. Bobby and I met, and I found him very down to earth, intelligent, and above all stoic. He was suffering from not only hemophilia, but HIV and hep C. I later became friends with his mother, who worked with me to help kids with hemophilia in Russia, something she had been doing for some time. Suzanne became such an expert on all things Russian, that President Ronald Regan relied on her as an advisor for Russian policy, and she welcomed him to Russia, to the Pavlosk Palace, which she had helped to renovate. Remarkable family!!

Bobby is also well known in Massachusetts for running for public office: he made history in 1994 by becoming the first person in the US to have HIV to run for Lieutenant Governor. He ran with Mark Roosevelt, a descendant of Teddy Roosevelt.

And now I just read over the weekend that Bobby has been cured of hemophilia. How? A liver transplant on July 10. If you never heard of this, factor VIII is produced in the liver. Transplanting a liver from someone without hemophilia will give you a liver that makes factor VIII. It’s only for life and death emergencies, though. The risks of it being rejected are too high, as are the risks of bleeding. Bobby needed it as his liver had been failing.

And true to his family heritage, he did it while making history. In a 10 hour surgery, he received a liver from someone who was receiving a liver from someone else… at the same time! A side by side liver transplant. This is called Domino Surgery, and this was performed at Emory University in Georgia.

We wish Bobby a good recovery! I am not sure but I think Journey, a marvelous book about hemophilia in the 80s and 70s, is out of print. Ask around: your local hemophilia chapter may have a copy you can borrow. There are limited copies available on Amazon, and eBay. It’s worth reading.

Book I am Reading
The Shack by William Young.

I bought this at an airport because A) I was desperate for reading material and B) It said Best Seller on the cover. It’s just not my style, I guess;  I could not get past the first few chapters. I skimmed through later the plot thesis: little daughter is murdered while family is on vacation (murdered in the shack), father Mack loses his faith, father receives a letter from God one day asking Mack to have a chat with him in the shack, Mack, who had turned skeptic, blindly says “Ok, sure, because maybe the letter is from God” and so on. So I stopped there; I guess I will miss the unfolding plot about how God is an overweight Chinese woman, Jesus is a lumberjack and I don’t even know what character the Holy Spirit was… maybe the postman? 

As an editor, a winced when the author writes how God calls a bluejay over to her. The bluejay first struts, then walks. Bluejays don’t walk like robins; they hop. The author didn’t do his homework, and being trained to look for these types of errors, I am my own worst enemy at trying to enjoy books!

Dan Dick, ordained minister of The United Methodist Church, writes: “The Shack is a spiritual Twinkie – sugary sweet with little or no nutritional value. The fantasy tale is very unevenly told, but framed as a might-have-happened second-person narrative. The spiritually naïve and immature might find this to be a deeply satisfying treat. Without a sound theological basis or the application of even the most basic critical thinking skills, a reader might mistake this as more than just a fairy tale.” Amen.

I won’t rate it given I didn’t read it it and will add that someone I know and respect very much read this, was moved about the message of forgiveness and subsequently reached out to a family member long lost and repaired a relationship. In that regard, this book has great value! It just wasn’t my spiritual cup of tea. 

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