Laurie Kelley

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.

True Crime and VWD: The Staircase Saga

Laurie Kelley October 6, 2024

Crime stories—whether fiction or nonfiction—have always fascinated but seem even more popular than ever. Murder mysteries and whodunnits. Organized crime or the kid next door. True crime—often about murder—ironically has new life recently, with smash-hits on Netflix like Lover Stalker Killer, The Menendez Brothers and Dahmer.

Our amazing archivist from North Carolina, Richard Atwood, researcher and reader extraordinaire, has a particularly interesting true crime story—the Staircase saga.

He writes: “This true crime event has maintained a bizarre fascination over two decades of debate. On the night of December 9, 2001, author Michael Peterson found his wife, Kathleen, dead at the bottom of a back staircase in their Durham, North Carolina home, surrounded by blood spatter. The police suspected murder and arrested Michael, who was the last person to see Kathleen alive. During the 2003 nationally televised trial, the prosecution introduced evidence that, sixteen years earlier, Michael was the last person to see a certain Elizabeth Ratliff alive before she was found dead—surrounded by blood spatter—at the bottom of a staircase in Germany. It was revealed that Elizabeth Ratliff had von Willebrand disease, a condition that complicated the determination of her official cause of death. There were too many coincidences between the two deaths involving staircases.

“This Staircase saga has personal connections. The murder and trial were a big deal here in North Carolina, as well as nationally. My son and his family live a half mile from the Peterson house. After Michael’s murder conviction was overturned and he returned to Durham, my son reported that friends of his would see Michael working out in the gym—a practice picked up in prison—then add slyly that it was on the step machine. This black humor is prevalent in Durham where many, but not all, consider Michael guilty of murder. I should also add that the psychologist named Margie Fargo, who was the jury consultant for Michael’s legal defense team, was my date to our high school senior prom in Atlanta back in 1968. Margie continues to profess Michael’s innocence.

“The body of work under the name ‘The Staircase’ keeps expanding. The 2003 murder trial was broadcast live and thoroughly discussed on Court TV, the former cable television network. The author Diane Fanning wrote Written in Blood: A true story of a murder and a deadly 16-year-old secret that tore a family apart (2005). The French filmmaker Jean-Xavier de Lestrade released his almost seven-hour documentary The Staircase (2005) on Sundance (and later on DVD). To provide updates, R.J. Parker wrote The Staircase: murder of Kathleen Peterson (2018) and Aphrodite Jones wrote A Perfect Husband (2019). As an author, Michael Peterson wrote Beyond the Staircase (2020), a slightly biased version of events that describe his prison experience. There may be other books about the murder trial, the overturned conviction after eight years imprisonment, and the subsequent plea deal. Then filmmaker Antonio Campos released the HBO series The Staircase: limited series (2022), The eight episodes, lasting eight hours, first shown on HBO, then released on DVD, star Colin Firth and Toni Collett, both accomplished actors. This fictionalized account of true events (filmed in Atlanta, not Durham) provides alternative explanations without stating a definitive explanation. It’s good entertainment.

“While I admire the acting skills of Firth and Collett, I don’t feel that they accurately portray Michael and Kathleen Peterson—he is not sour enough. I’m also a fan of Juliette Binoche, who plays the documentary film editor.

“But my biggest complaint about all the Staircase contributions is that von Willebrand disease does not get enough attention. Dr. Henry Lee had an excellent expert witness explanation of blood spatter during the trial. And then there is the ‘owl theory.’ I have an owl that perches on a branch overhanging my Winston-Salem yard most days. He is peaceful, unlike the alleged ‘killer owl’ in Durham, 75 miles away. Michael sold the house in Durham to pay his legal bills – the new owners spent a fortune fixing up the house on four acres. Margie Fargo lives in Charlotte; I saw her at a high school reunion in Atlanta. Michael continues to live in Durham, though I haven’t heard whether he still works out at the gym; he is close to 80 now.

“If you want some good true-crime reading, or wish instead to binge watch, then consider something from The Staircase saga – and be like me, complaining that von Willebrand disease should get more attention.”

Busted!

Laurie Kelley September 29, 2024

It was bound to happen. After 28 years of hand-delivering factor to foreign countries, I had to forfeit my gifts to patients and surrender the factor to customs. I traveled to Tanzania in early September for a vacation—it’s one of my favorite places in the world to visit. I was excited to introduce my boyfriend Doug to Africa, as it was his first trip. We would also travel to Rwanda afterwards, for a gorilla trek, but also to deliver factor IX to a little boy I’ve known for ten years.

But… customs had other ideas.

Things have changed radically in the world since I started delivering factor in 1996. I usually don’t bring paperwork with me. I provide paperwork when I FedEx factor—and let me rave about FedEx which has been so good to us. FedEx just seems to go everywhere and our factor has gone to Christmas Island, Iraq, Afghanistan… you name it. But when I travel with factor, I just stick it in the check-in luggage and hope for the best.

I’ve been single-out before: Argentina for a World Federation of Hemophilia Congress. Jamaica. Russia. Pakistan (65 pounds of factor, in fact). But customs has always let me go when they hear why I am bringing this life-saving drug into their country—because their government doesn’t provide it for their suffering citizens.

But times have changed. Security cameras are everywhere and even when a custom official hears my sad story, they can’t unsee it, and neither can the cameras. I lost about $58,000 in factor, and had to pay a hefty fine on top of surrendering the factor.

I felt so sorry for the little boy in Rwanda. I showed up empty-handed. Supplies of factor IX for donation are drying up. WFH donations are becoming less common for FIX; so many patients ate appealing to me for FIX. There simply isn’t enough to go around. While we push national organizations to get patients registered, which boosts the number of known patients, we don’t have enough factor for all, despite this being the rallying cry of the community.

Well, we had to look at the humorous side to it. Of all the boxes I chose, that I keep in storage for shipping, what did I pick? A pretty blue box that must have held perfume, lingerie or something else that was risqué. The lettering on it said, “Tempt Me” and customs took that much too seriously.

When Can an Inhibitor Be Diagnosed?

Laurie Kelley September 22, 2024

Inhibitors are relatively uncommon. If one develops, it’s usually detected when a child with hemophilia is young, usually in the first one to three years of life. The median time to development of an inhibitor is about nine to eleven exposure days— days on which the child received one or more infusions of factor VIII or factor IX. Parents may suspect that something is wrong, usually because a bleed will not resolve, or takes significantly longer to resolve, after an infusion of factor. Several situations might indicate that a child has an inhibitor:

Unresolved bleeds

All HTCs routinely monitor their patients for inhibitors, and many do so more frequently in the first months of treatment. But not every child with hemophilia is seen at an HTC, and older children may go only once a year for their annual comprehensive visit. You may learn that your child has an inhibitor the hard way: when factor no longer works well to stop bleeds. How do you know when factor isn’t working well? If you’re new to hemophilia, this may not be easy—you may not be sure how long it should take a bleed to stop after an infusion. You’re still getting used to identifying symptoms of different bleeds, learning about your child’s individual bleeding pattern, and determining whether a “normal” muscle or joint bleed takes one day or four days to resolve. With so much to learn, it may not immediately dawn on you that the infused factor isn’t working properly, and a bleed is taking much longer than it should to heal. It’s common for parents to think that they simply need to give their child more frequent infusions, or give a higher dose per infusion. Continued bleeding, even with repeated infusions of factor, is the primary way that parents discover their child has an inhibitor.

Increased bruising

Bruising in young children with severe hemophilia is common. But if your child uses factor frequently and you notice increased bruising, this may be a sign of an inhibitor. One mother commented that her doctor noticed increased, repeated bruising at her child’s infusion site, and suspected an inhibitor.

Routine clinic visit

Ideally, you’d like to learn that your child has an inhibitor before an emergency happens and before he gets a major bleed. A blood test at your child’s HTC comprehensive clinic visit can identify an inhibitor. It’s wise to have a child with hemophilia tested for inhibitors routinely: learning that he has an inhibitor in advance of surgery or a major bleed allows parents to have a plan in place and the correct treatment on hand.

Breakthrough bleeds while on prophylaxis

Prophylaxis is the scheduled infusion of factor to help prevent bleeding. Many children with hemophilia on prophylaxis receive factor two to three times a week, enough to keep the lowest level—called the trough level—of factor in the blood above 1%. Children on “prophy” usually will have enough circulating factor to prevent most spontaneous bleeds and abnormal bruising. When a child on prophy starts bruising or even bleeding more often than usual, an inhibitor may be inactivating some of the factor, thus lowering his factor level and increasing his risk of bleeding.

Bleeding after surgery

Because surgery often involves blood loss, it requires special precautions for children with hemophilia. A common first surgery is circumcision. In families with no history of hemophilia, elective circumcision is usually performed soon after birth, and prolonged bleeding that results from the surgery may indicate hemophilia. A blood test will confirm the diagnosis. Depending on the circumcision method, some babies with hemophilia have no bleeding problem with circumcision—even without an

infusion of factor. Others bleed profusely and need factor to control bleeding. Any kind of surgery on a child with hemophilia requires careful planning and monitoring of factor levels, and any child who continues to bleed following surgery, even with adequate factor, should be immediately tested for an inhibitor. Ideally, all children with hemophilia should be tested for an inhibitor before any surgery of any kind.

Allergic reaction following infusion

Inhibitor development in children with hemophilia B is rare—only 1% to 3% of people with hemophilia B get them. Unlike people with hemophilia A and inhibitors, about 45% of people with hemophilia B and inhibitors also develop allergic reactions at about the same time that they develop inhibitors. Allergic reactions may start out mild, and then increase in severity after repeated exposure to products containing factor IX, often to the point of a serious, life-threatening allergic reaction called anaphylaxis. Allergic reactions in hemophilia B patients are not in themselves an inhibitor, but are a sign that an inhibitor has developed.

Later in life

If an inhibitor is going to develop, it will usually do so within twenty exposure days, and almost always before the hundredth exposure day. And the patient will probably have severe hemophilia. Only about 1% to 2% of people with mild hemophilia develop inhibitors. Although most inhibitors occur early in life, in rare cases an inhibitor develops when a child is older, sometimes a teen or an adult. Unlike people with severe hemophilia, people with mild hemophilia who develop inhibitors tend to develop them later in life, usually after intensive exposure to factor during and after surgery or traumatic injury.

Regardless of how you discover your child’s inhibitor, the diagnosis can be both a relief—now you know what’s wrong—and a worry. What will happen next? Your HTC is the best place to turn to for treatments to help manage the inhibitor.