Laurie Kelley

Birthplace of America’s Hemophilia

Laurie Kelley November 24, 2024

It’s Thanksgiving week in the United States, and living in the Boston area, one cannot help but think of how our country was first founded, and by who. When I travel just 15 minutes from my house to Newburyport, Massachusetts, a lovely seaport city, I can read plaques on the houses that read “1820,” “1775” and even “1656”! Many of these houses have existed since before America was born! Boston is the birthplace of our country, and Massachusetts is naturally a state of many firsts.

It was in Plymouth, Massachusetts that the Puritans landed; and here that the settlers weathered the severe first winters with help from the native tribes. And it was here that the first person with hemophilia was born in the US! In fact, I can travel 15 minutes from my house and see where he lived.

I run the following article almost each year, so we can remember this famous “first” family in the US: enjoy!

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 Appletons in 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 Parent Empowerment Newsletter, November 2002

“THE APPLETONS: America’s “First Family” With Hemophilia” by Richard J. Atwood and Sara P. Evangelos
© 2002 LA Kelley Communications, Inc.

The Philippines: Unseen Forces of Support

Laurie Kelley November 10, 2024

My name is Angelo, and I am living with hemophilia in the Philippines. This condition has been a constant companion in my life, presenting unique challenges that not everyone fully understands. Hemophilia affects how my blood clots, making even minor injuries a potential crisis. This is my story of resilience, hope, and the invaluable support I’ve received from people who truly care.

Despite all the care and infusions, the aftermath of those hospitalizations left my veins delicate, making IV insertions a struggle. Even a 22G cannula, a larger-than-average needle, now poses challenges, often requiring multiple attempts to find a usable vein. I recall one hospital admission where a series of failed attempts to secure a line left me feeling exhausted and stressed. It was a wearisome experience, physically and mentally. Fortunately, my compassionate hematologist at the time understood my situation and allowed me to go home after a few successful transfusions, even though my hemoglobin levels were still below ideal. This was a small victory in a long and difficult battle.

Since my last hospitalization in 2017, I have managed my condition at home. I often choose to bear the pain of bleeds rather than face the struggles of another hospital admission. The thought of being poked and prodded again is daunting, so I’ve learned to cope with my condition as best as I can. My condition has also affected my mobility, I require crutches for support, relying heavily on my upper body to assist me in standing. Both of my feet, especially my right one, are significantly slanted and cannot lie flat on the floor, complicating my ability to move around. My knees, particularly my right knee, are severely restricted in movement, adding yet another layer of complexity to everyday life. Simple tasks like walking or even standing for long periods can become exhausting, and I often have to plan my day around my physical limitations.

Despite these struggles, I am deeply grateful for Hemophilia Advocates- Philippines (HAP), our hemophilia organization here, which has been a lifeline during emergencies. Their support has been crucial in moments when I needed help the most. Over the years, I have requested assistance for factor multiple times, with my most recent request made last October. Unfortunately, it was not granted due to low supplies that are only allocated for emergencies. While it was a challenge, I remained hopeful, knowing that support comes in many forms. In light of the situation, I turned to Ma’am Laurie Kelley, a compassionate individual known for her generosity. I shared my situation with her, and to my relief, she kindly offered to send me factor herself. Her willingness to help reminded me of the goodness that still exists in the world.

Living with hemophilia in the Philippines, where medical assistance for rare bleeding disorders is scarce and costly, has been a constant challenge. The healthcare system here is often strained, and access to specialized treatment can be limited. Although I am employed, the average salary here makes it incredibly difficult to afford the necessary treatments on my own. I sometimes worry about the future and how I will manage my condition without the support of others. Yet, through the relentless support of people like Rev. Fr. Kill, Ma’am Laurie, and HAP, I continue to find strength and hope. Their kindness has taught me that I am not alone in this fight.

This journey has taught me to appreciate every act of kindness and to stay resilient. I have learned that, while my condition presents many challenges, it has also brought extraordinary people into my life. For those of us with rare conditions, even small acts of understanding and support can make all the difference. It’s these connections that give me hope for a better tomorrow, where I can live my life with dignity, strength, and the support of those who care.

Disaster Scenario

Laurie Kelley November 3, 2024

Valencia, Spain was ripped with a horrific flood this week, comparable to the effects of a tsunami. At least 205 are known dead and the count will surely climb. In the US, we recently had Hurricane Milton, one of the deadliest storms to hit Florida on record. Recently there were also floods in Nepal and the Sudan, and a hurricane in Mexico and again, Florida.

It’s a reminder to be prepared for disaster scenarios. These disasters are promoted on the news frequently, and may point to climate change as storms seem to be more intense and severe. Whatever the cause, there are many things you can do as a bleeding disorder patient and family to be prepared for catastrophe.

First, know the geography and weather patterns wherever you live. California has many geographical faults that cause earthquakes; wildfires are particularly bad. The midwest states of Texas, Nebraska and Kansas are dubbed “tornado alley” for their frequent and sometimes devastating tornadoes. The northeast has “nor-easters” every ten years or so. And Florida, as we’ve seen recently, is hit with hurricanes that often originate off the coast or in the Caribbean Sea.

On the east coast, we know that September and October are hurricane season moths. Normally we are lashed with the tail end of a Caribbean-originated hurricane. We once had “The Perfect Storm,” a 1991 hurricane that struck on October 28, and became a massive killer. Damage was most severe in Massachusetts; the story became a movie of the same name, starring George Clooney. We ensure we are stocked with wood for a fire, shovels, spare food—even snowshoes!

Second, always ensure you have enough factor and ancillaries. When storms hit, you often are not able to travel, and face power outages. Your factor supplier may not be able to bring you factor, and you may not be able to contact them. Keep at least 10-20 doses in your fridge at all times, and plenty of ancillaries.

Third, keep candles and flashlights ready, with fresh batteries. Keep your cell phone charged, and have a power bank charged and ready if your phone runs low on power.

Finally, know your local emergency numbers, in case you need to be evacuated due to your medical condition. Callers have overwhelmed 911 services, so you may not be able to always reach help when you need it.

As soon as you hear that a storm is developing, even if it is in Florida and you are in Massachusetts, be prepared for a worst-case scenario. Keep updated with popular apps like RadarOmega, Radarscope, and the FEMA App. Ask your local bleeding disorder organization what they suggest for being prepared and for help during a natural disaster.

Don’t wait till disaster strikes! Don’t be caught unprepared.

Pain and Gain

Laurie Kelley October 20, 2024

Each October 16 I give a silent thanks to one of the Massachusetts men who helped to eliminate pain in surgery: Dr. William Morton (1819-1868). He was born in Charlton, Massachusetts, where my grandmother grew up.

As a dentist, he witnessed the incredible pain his patients endured. During his time, there were no pain killers for surgery. Medicine was just at the beginning of becoming the profession as we know it today. Even as late as 1874 in the US, medical schools were little more than trade schools. Applicants didn’t need undergraduate degrees. There was no consensus on treatment, no models for medical studies. Most physicians still followed the Hippocrates theory of the “four humors.”

Disease and deformity were rampant, especially in the overcrowded and teeming cities of the world. The cause of infection was not discovered yet; there was no understanding of germs. And the pain of surgery was intolerable. Limb amputation most common type of surgery and the “best” surgeons were the fastest: the best could remove a limb in five to ten minutes. But blood transfusions were not available yet, and patients often went into shock and died. It’s no surprise that prior to 1846 little elective surgery was done.

All of that changed on October 16, 1846. Morton first publicly demonstrated the use of inhaled ether as in surgery at Massachusetts General Hospital, in the now named “Ether Dome.” Just two weeks prior, he had performed a painless tooth extraction on patient Ebenezer Hopkins Frost, using ether. On October 16, using Morton’s ether, Dr. John Warren removed a tumor from the neck of a patient—without pain. The news spread like wildfire around the world. Oliver Wendell Holmes—physician, lawyer, poet, and founding father of the Supreme Court—dubbed it “anesthesia.”

The medical breakthrough was not without its controversy. Correct dosages were not yet known—that would be experimented with by Dr. John Snow of England. Morton’s partner, Dr. Wells, objected to Morton claiming to be the only one to have discovered this, and trying to patent it. Their partnership never recovered. The patent was never enforced, and ether soon came into general use.

Morphine had been first isolated between 1803 and 1805, and Merck began selling morphine commercially in 1827. With the invention of the hypodermic syringe in 1853, morphine use spread. Localized anesthesia began to be used in 1884 by injecting cocaine into the wound location. Pain was relieved, but addiction, even among doctors, spread.

MGH honors the October 16 event in its Ether Dome, which is open to the public. Between 1821 and 1868, more than 8,000 operations were performed there. Today it is a teaching amphitheater and historical landmark. Visitors can appreciate the architecture and study the artifacts related to the elimination of pain, so important to those suffering, like our patients with bleeding disorders.

Before whom, In all time, Surgery was Agony

By whom, pain in surgery was averted

Since whom, science has control over pain

— Morton’s tombstone, Mt Auburn Cemetery, Cambridge, Massachusetts

Writing Your Autobiography

Laurie Kelley October 13, 2024

Have you ever thought that your experiences with a bleeding disorder would be worthy of a book? That others would be educated, comforted, even entertained by your experiences?

Many have. And they’ve written their own story—an autobiography. I believe the first commercially published account was the classic Journey, by Robert K. and Suzanne Massie. For many parents in my age group, it was our first read about hemophilia, and it was scary. Of course, it detailed a time of raising a child with hemophilia in the 1960s, when treatment consisted of constant trips to the hospital to get plasma or cryoprecipitate. But it is expertly written.

It took a while for other autobiographies to follow, but eventually they did, concerned mostly with the HIV scandal. Eventually, other books followed, including stories about life with modern treatments, included children’s books (told “by” the child), books about athletes with a bleeding disorder, and more.

Unlike Journey, many of the books are self-published. This is a fast way to publish a book but risky. Many of the books are not properly edited. Some have typos, or lack organization. As I’ve been publishing books since 1990, and have a great editorial team, I’ve been approached by some people to help them write their autobiography. I offer my editorial services for free, but there are times when I will reject a story because of lack of focus, poor writing and authors who don’t want to accept advice.

I recently read a book by one of my favorite authors, Ray Bradbury. Zen in the Art of Writing is an easy book to read, full of practical tips, and motivating. Bradbury is a master. If you think you have a story good enough for an autobiography, consider buying and reading this great book as a start. At the very least, consider his sage advice. He emphasizes having passion, exercising your reading and writing skills (the first step in becoming a writer is to be a voracious reader) and be brave!

From the Master himself:

The first thing a writer should be is excited. What do you want more than anything else in the world? What do you love, or what do you hate?
Be passionate with abandon. Tomorrow, edit and cut. But today “explode—fly apart—disintegrate!”
Write fast at first. The faster you write, the more honest you are. Bradbury wrote the draft to his masterpiece Fahrenheit 451 in only nine days.
Practice writing—anything. Bradbury wrote at least a thousand words a day, from age twelve on.
Don’t always look outward for ideas, look inward. Think, dream. Ponder your life. Tae long walks and let thoughts flow.
Read poetry every day. Poetry is different than prose, and causes us to think differently, more creatively, which will help your writing.
Read daily; find an author whose writing you like and can learn from.
Use all your senses when you write: color, sound, taste, and texture.
Edit! Find a good, professional editor and learn yourself how to cut. Bradbury writes of the author, “His greatest art will often be what he does not say, what he leaves out, his ability to state simply with clear emotion, the way he wants to go.”

And drum and crack the mind to awe,

To behold beauty yet perceive its flaw.

Then, flaw discovered, like fair beauty’s mole,

Haste back to reckon all entire, the Whole.

This done, pretend these wits you do not keep,

Go panther-pawed where all the mined truths sleep.

—Zen in the Art of Writing, page 157.