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America’s “First Family” With Hemophilia

Laurie Kelley February 22, 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.

New Alex Lieber Memorial Scholarship

Laurie Kelley February 8, 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 devastated 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: https://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, which 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. 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.

Happy New Year!

Laurie Kelley December 28, 2009

The staff at LA Kelley Communications wishes everyone in the hemophilia and bleeding disorders community worldwide a very happy new year.

This past year we witnessed too many pass away from untreated bleeds and we pledge to work even harder in 2010 to continue to help those in developing countries with bleeding disorders. Our deepest appreciation to all who help us in this noble endeavor!

A Christmas Gift of Life

Laurie Kelley December 21, 2009

Bob Massie was given a second chance at life this summer. In a previous blog I shared 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 person with HIV 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 IU 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. 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, and Al Gore.

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 his 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 well. 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.

Wilate: a New VWD Product

Laurie Kelley December 14, 2009

This is big news for von Willebrand Disease patients: there’s a new product on the market. Wilate® just got approved by the US FDA for “treatment of spontaneous and trauma-induced bleeding episodes in patients with all types of von Willebrand disease (VWD),” according to a press release. Wilate is a high-purity plasma-derived product, which uses a double viral inactivation process in manufacturing: solvent/detergent (S/D) process and a special terminal dry-heating (TDH) system. No albumin is added as a stabilizer. Wilate is exclusively derived from large pools of human plasma collected in U.S. FDA approved plasma donation centers. Wilate will be available in the market in early 2010.

Wilate is produced by Octapharma, a Swiss-based company. This is the first time Octapharma has entered the US bleeding disorders market. Wilate will be in direct competion with Humate-P, manufactured by CLS Behring.

One thing interesting I learned is that this is the first product developed and manufactured specifically for VWD.

Here’s a quote from Octapharma: “Octapharma’s worldwide commitment to coagulation disorders dates back to Octapharma Group’s formation 25 years ago,” said Octapharma USA President Flemming Nielsen.”We are thrilled that U.S. patients will now have access to Wilate following its significant success in Europe as a next generation therapy. Octapharma is committed to providing the U.S. market with life-enhancing therapies.”

VWD patients now have more choices for treatment options. For more information, please visit www.octapharma.com.

Great Book I Just Read
Final Voyage: A Story of Arctic Disaster and One Fateful Whaling Season by Peter Nichols

Massachusetts (my home state) gave birth to the American Revolution and also the global whaling industry. This fascinating book recounts a terrible story of scores of whaling ships trapped in the Arctic region, as the captains tried to score as many whales as possible before the ice closed in for the winter. Nichols deftly parallels that story with the beginning of the whaling industry, which has direct ties to Puritans coming to the new world, religious intolerance in Massachusetts in the 1700s, and the Quakers, who almost single-handedly created this lucrative industry. Whale oil soon was lighting the homes of Massachusetts and then lubricating the machines of the Industrial Age, making some Quakers millionaires, even then. Nichols also details the decline of the industry: the discovery of crude oil in Titusville, Pennsylvania, which soon replaced whale oil; the decimation of so many whales left hunting lean. All these events culminate into a dreadful outcome for the ambitious captains of the whaling ships that last season of hunting, as they searched for the last whales in an early and fearsome storm. This book will enlighten you about many subjects, from religion and commerce, to whales and history. Three stars.