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Sunday, February 28, 2010
An 8.8 earthquake rocks Chile this weekend--a second major earthquake in a developing country in only 6 weeks. So many people asked about those with hemophilia in Haiti, that I'd like to reassure everyone that Chile has a very good hemophilia health care system, and those with hemophilia should be well cared for, even following the quake.
And I have good news about Haiti: we learned that one boy we know there, who has factor VIII deficiency and whom we have helped before is okay, and we are sending more factor to him. This summer I hope to visit Haiti to get a patient registry started, to learn where hemophilia patients are and how many there are. Following the earthquake, so many NGOs are teaming together to bring help, that I will piggyback off some groups already established there, who have pledged to help us.
This past week I was in New York City (yes, during that terrible storm!) and visited the ambassador to the United Nations from Zimbabwe, His Excellency Boniface Chidyausiku. This is the UN dignitary who helped us speed up Elton's visa approval one year ago, so Elton could come to the US for emergency surgery at RUSH Hospital in Chicago. It was a wonderful meeting, and we reminisced about visits to the beautiful country of Zimbabwe and the people we both know in health care there. Accompanying me was Juliet Hanlon, UN Goodwill Ambassador and recording artist, who made this meeting possible.
There are good things happening internationally, despite the often sad news of political unrest or natural disasters. Even if it's a story of one boy from Zimbabwe or Haiti, it's enough to keep hope alive.
Good Book I Just Read
The Secret by Rhonda Byrne
I was given this book over a year ago and finally read it, in two nights. A little gem! There's nothing new presented here that Napoleon Hill didn't already present previously in his landmark Think and Grow Rich or that Wayne Dyer hasn't presented for 20 years now in his books and on public television. But this sure is marketed and packaged in a way that makes you savor it. The book is about the Law of Attraction, and how the universe will provide if you make your intentions and desires clear and visualized. Mankind has known about this Law for eons, and I've read many books on the subject, but I really enjoyed how this one was set up and presented. It gets a bit hokey a few times, and I didn't like the heavy marketing of the DVD, but overall, a light read, one that will make you feel wonderful and I do believe it works! I've used it myself with success. Three stars.
Sunday, February 21, 2010
Last week I wrote about the most famous carrier of hemophilia, "Young Victoria," and that got me thinking that we have our own royalty, America's first family with hemophilia, the Appletons. This article is from a 2002 issue of PEN, and well worth reading! And, I live about 15 minutes away from the family farm!
New England, 1639. Imagine that you are standing on the deck of the sailing
ship Jonathan. You have just glimpsed the shore of your new home, the Massachusetts Bay Colony. Imagine the brilliant New England foliage, the bright chilly wind. Imagine your dream of farming your newly acquired land. Imagine the adventure. Now, imagine that you are the first European with hemophilia to step on the North American shore.
John Oliver (1613–1642) traveled from Bristol, England with his family to settle under the leadership of the Massachusetts Bay Company. He lived for only three years after he reached North America, fathering one child, Mary, and dying young as a consequence of his hemophilia. Not until after 1800 did the medical community begin using the term hemophilia to describe his disorder. John’s daughter, Mary Oliver (1640–1698), was likely the first hemophilia carrier of European descent born in the colonies. With her husband, Major Samuel Appleton, Jr. (1625–1696), Mary had three daughters and five sons. One of these sons, Oliver Appleton (1677–1759), was the first American colonist born with hemophilia.
Early Ipswich Roots
Mary and Major Appleton lived in a settlement known to native Americans as Agawam, but re-christened by the English
in 1633 as the town of Ipswich. What would life in Ipswich have offered their son, Oliver Appleton? Thirty miles north of Boston
on the Atlantic shore, Ipswich was owned by the Massachusetts Bay Colony; it was purchased earlier in the century from Native
Americans for 20 British pounds. By the mid-1600s, Ipswich ranked second only to Boston in population and wealth. The Appletons were a wealthy colonial family. Major Samuel Appleton, Jr., Oliver’s father, was the son of Samuel Appleton Sr.,
one of the “landed gentry,” and a good friend of John Winthrop, the first governor of the Massachusetts Bay Colony. Appleton’s
fertile 460 acres of farmland had been granted to him by the Colony in 1638, and left to his son, Major Appleton, around 1670.
Major Appleton, who served as a judge at the infamous Salem Witch Trials in 1692, died in 1696. He left his now nearly 600 acres, split into four parcels, to his four sons: Oliver, Isaac, Samuel and John. Oliver’s 100-plus acre inheritance included his father’s sawmill, ox pasture, and farmland bordering his brothers’ parcels.
In 1701, Oliver married Sarah Perkins. Well-to-do millers, farmers and traders, Oliver and Sarah possessed numerous household and farm goods. They were involved in local politics, church affairs and business. Together they raised fourteen children; several sons and their descendants would become fine cabinetmakers. At the turn of the eighteenth century, Oliver and his three brothers were working their adjoining farms in a loosely communal style. Each brother might grow a crop that the other brothers could use. Yet each brother farmed separately, produced his own goods for trade (like basket hoops), and kept his own business ledger. The brothers owned cattle, sheep, turkeys and hogs, and traded goods with family and friends in Ipswich.
A Dangerous Occupation?
On their “new” land (already cleared and cultivated by Native Americans), the Appletons cut and milled timber, raised livestock and worked the farm. Today, farming is still one of the most dangerous occupations. In the seventeenth and eighteenth centuries, its hazards were surely compounded by Oliver’s hemophilia, and the harsh New England winters. Yet Oliver lived to be 82—a considerable age in any century.
Late in life, Oliver was confined to his bed and developed bedsores on his hips. At age 82, his cause of death is recorded as bleeding from his bedsores and his urethra. Oliver appears to have been a generous and fair man, dividing his estate equitably
among his children and his wife Sarah.
Making Medical History
Oliver and Sarah had six daughters and eight sons. Two of the daughters, Sarah and Hannah, had sons with hemophilia.
Interestingly, Hannah’s sons, Oliver and Thomas Swaim, were doctors. What would they have thought of their family’s disorder?
Without letters or other documents, we can only guess. Yet it was the Swaim branch of the Appleton family that attracted the attention of the medical community. Based on his personal connection with the Swaim family, Dr. John Hay, a Massachusetts physician, published an article on the Appletonsin a New England medical journal in 1813. Following this publication,
the Appleton family history appeared in numerous medical journals, at least as late as 1962. By then, the family had been traced through 350 years and 11 generations: 25 males with hemophilia, and 27 carrier females. In 1961 a blood
sample, drawn from the last known living carrier in the family tree, revealed factor VIII deficiency, or hemophilia A.
Are the Appletons America’s “First Family” with hemophilia? Perhaps, in the sense that our knowledge of hemophilia has
been enriched by the study of this large and long-lived colonial family. Thanks to our American Revolution, we have no “royal
family” with hemophilia. Yet we can still honor and remember the Appleton family. This Thanksgiving, we can recall the challenges faced by earlier generations with hemophilia—people who contributed to our heritage as Americans, and as a hemophilia community. To understand ourselves, and create our vision for the future, we must always remember the past.
From the November 2002 Issue Parent Empowerment Newsletter
THE APPLETONS: America’s “First Family” With Hemophilia
by Richard J. Atwood and Sara P. Evangelos
© 2002 LA Kelley Communications, Inc.
Good Book I Just Read
Anthem by Ayn Rand
Now, you must know many Teabaggers are citing author Ayn Rand and her philosophy at meetings, on blog sites, etc. Politics aside, I happen to like Ayn Rand a lot. I decided to read this little gem, published in 1947. A daring, thoughtful, if often campy piece, it is a good vehicle for a discussion on Rand's theory of objectivism.
Anthem is a diary by someone who is in a society of the future, where apparently communism reigns. People are told when to mate, are separated from their babies, told what they will be when they mature. And the word "I" does not exist. Some cataclysmic event wiped out civilization and humankind starts over, with communism at its core. One man, "Equality 7-2521," realizes he is different and despite being warned not to be, is curious, daring and ambitious. He wants out. One day while acting in his role as street sweeper, he finds a tunnel, which leads to an underground world long forgotten, and every night, sneaks away to explore. Little by little he uncovers the secret of humanity's past.
A quick read (I finished it in 90 minutes) it has plenty to think about and discuss in book clubs. It's a bit dated of course, but it's still worth a read! A forerunner to the TV series "The Prisoner," I suspect. Three stars.
Sunday, February 14, 2010
I went to see the movie "Young Victoria" yesterday, which chronicles the most famous hemophilia carrier's 1837 ascension to the throne as just a girl of 18. It also tells of her meeting with and eventually love for Prince Albert of Belgium. Lavishly filmed, I felt as though I had gorged on Godivas, taking in the costumes, scenery and gold. I thought the performances by Emily Blunt as Victoria and Rupert Friend as Albert were understated and well done. It was a very enjoyable movie. I was surprised it was such a love story, and the timing was perfect as it was a day before Valentine's Day.
We always seem to think of Britain's longest reigning monarch as a blimpy old woman, but here, she is beautiful, confident and headstrong. As you watch the courtship of the Princess and Prince, you marvel at how much they loved one another (by all accounts they truly did), and also by their tremendous wealth and privilege: Victoria cannot even walk down the stairs by herself, lest she fall. She always needs to hold someone's hand (she abolishes that practice when she becomes queen). Despite the young love, and their wealth, one thing remains unsaid but we all know it happens: Victoria and Albert eventually have a child with hemophilia, Leopold.
I watched the ornate movie, and the tender love, and felt so sad for them! Like us, this young couple would have a child who suffers, but unlike us, there was no treatment. It was really poignant to see this beautiful young couple, blindly in love, happy, not knowing what fate had in store for them.
Victoria and Albert had nine children: three girls who were carriers and of course, Leopold. Eventually, she passed hemophilia on to the Russian, Spanish and German royal families. This is why hemophilia is called the "royal disease." She had 42 grandchildren!
Albert died at age 42, after 22 years of marriage. The movie ends with a title which revealed that Victoria had his clothes laid out every morning for the next 40 years, in his memory. She forever wore black following his death. A movie worth seeing, especially if you have hemophilia in the family.
Great Book I Just Read
Child of the Dark by Carolina Maria De Jesus
This may quite possibly be the best book I'll read all year. I don't think I'll ever read another book like it.
Carolina Maria De Jesus (1914-1977) lived in the favela, the slums of Sao Paulo, Brazil, surviving only one day at a time by picking up paper and scrap metal and selling it. Intelligent and single, she kept a journal, which became this book. She writes so much about hunger, the horror of slum life, rummaging through garbage to find something to feed her three children, and always hoping for a better life. Her insights are amazing and her description of the slum is simple but painfully portrayed. I couldn't put this book down. I myself have been through slums in Central America, Brazil, Kenya and India. I often think that we could not live one day in the shoes of the poor, and after reading this book, I know it is true.
As amazing as her journal writing is, a co-author provides an epilogue: what happened after her book is published. Caroline, illegitimate and poor, rockets to fame as she becomes Brazil's best-selling author. This book is a must read! Four stars.
Sunday, February 07, 2010
I am very pleased to announce that we are offering a new scholarship for young men with hemophilia or anyone with von Willebrand Disease. And best of all, we are offering this as a way to help pay for books, travel costs, dorm living... anything that the student needs to help get them through a semester. The scholarship will not be given as a payment to the college, but as American Express cards in the amount of the scholarship. This will help the student practically and they can be used immediately.
We've tried to make it simple. The candidate should have decent grades and of course a bleeding disorder, but what we are really looking for is someone with compassion and initiative, someone with solid volunteer experience.
This is important because it reflects the values of the young man we seek to honor through this scholarship, Alex Leiber of Texas. Alex was only 16 when he was struck with bacterial meningitis. He died on December 21, 2003, just four days before Christmas. Within just 48 hours, he was gone, leaving behind a heart-broken family and many friends who have kept his memory alive. You can read about Alex and what a remarkable young man he was on the scholarship page. Stories about Alex were actually submitted by his mom Tammy to me when he was just a child, for the first edition of my book Raising a Child with Hemophilia, back in 1990. Like everyone, I was devestated to hear we had lost him.
We are so proud to be able to offer this scholarship to honor Alex, and thank Tammy and Jim Lieber for allowing us this privilege. The Alex Lieber Memorial Scholarship is offering two $1,000 scholarships. Persons with hemophilia or von Willebrand disease accepted to an accredited college, university, or vocational/technical school can apply. Candidates must be able to demonstrate a strong record of volunteer service.
The deadline is April 1, 2010. You can apply here: http://www.kelleycom.com/AlexLieber/application.php
We hope to hear from you! And God bless Alex and his family, now and always.
Interesting Book I Just Read
The Story of Edgar Sawtelle by David Wroblewski
This is an intriguing fiction, about a mute child growing up in a Wisconsin family that breeds dogs, dogs so well matched genetically and trained that they command premium prices. The story is about love and disappointment, family members and dashed expectations, and loss. Much loss. Edgar's muteness gives him a special bond with his dogs, whom he learns to train. The father and mother love Edgar, and their dogs. But tragedy strikes when Edgar's beloved father dies suddenly. And herein lies the trouble for me... the book, so lyrically written, with such amazing prose, and imagery that will have you even put the book down and wonder how someone could write like that... just seems to then take a Hollywood cookie-cutter approach. I suddenly saw this story, which spends an awful lot of time with tremendously detailed background about dog breeding, and the family's own roots and members, and presents deep psychology, become, well, like a screenplay. A whodunit.
And there are multiple comparisons that could be made to the Jungle Book (both Wroblewski's and my favorite childhood book) and Hamlet. Well, is it Mowgli or Hamlet? Pretty different comparisons. There are even allusions to Colton Harris-Moore, the barefoot burgler kid of Camano Island (the one who steals planes). Some characters are just not explained well at all. Others, like the amazing character of Almondine the dog, are brilliant and consistent. The book is over 500 pages, not an easy read for most, and after I realized it was going to be a whodnit with a denouement that I can just see up on the big screen, it kind of lost its charm for me. I found myself just flipping pages fast to get through it. (And let it be known I devoured every page of Moby-Dick) The writing, however, is superb. My daughter is now attending the college that Wroblewski attended, and she's a pretty good writer herself. I know this is a good book (rave reviews from most critics), but despite the masterful imagery, and an unforgettable central character in Edgar, I just saw a sell out somehow; it started so fresh and wondrously creative, then kind of nosedived into this ridiculous Columbo-style plot (although Columbo was better). The ending is unbelievably depressing. But it's still worth a read. Two stars.
Monday, February 01, 2010
There was lots of news this week under the heading of "What can I look forward to when I don't have gene therapy?"
How about longer acting factor? So, instead of prophy three times a week, you dose once, and it lasts all week? Long-acting factor is the next great thing in hemophilia, while we wait someday for a cure. I think this will be a reality in the next two years.
This week Biogen Idec (a company right here in Massachusetts; yes, they are freezing just like the rest of us this week) announced that they've dosed their first patient with long-acting recombinant factor IX.There are about 6 companies studying long-acting factor IX. One of these is also GTC Biotherapeutics, also located in Massachusetts, which has a FIX product in preclinical studies. And this is really different: GTC is using transgenic animals, like rabbits and goats. These animals are specially bred to excrete human FVIII or IX through their milk glands. Fascinating!
Now even when a company has a new product in clinical trials, this doesn't mean that it automatically will come to fruition. These are trials, and sometimes things don't work out. for example, just this week Bayer cancelled its LipLong study, which seemed so promising. Bayer was exploring a long acting factor VIII (BAY 79-4980), but results weren't as expected. But don't worry; they are also working on another long acting FVIII in different preclinical trial!
One thing to note: you can actually voluteer to participate in one of these studies. Go to clinicaltrial.gov. You could help make history!
Great Book I Just Read
Take Yourself to the Top by Laura Fortgang
I read this book every two years at New Year's to get motivated, to reasses and to plan. Not only for my business, but also for myself. This book has been around a while, but I love its no-nonsense approach, its friendly and cajoling tone, its practical applications for thinking bigger, laying the groundwork for success and removing all obstacles. I recommend it for anyone starting a business or starting a new passion within a business. First step, get off the caffeine. Man, it's like she reads my mind in this book at times! Four stars.
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