Laurie Kelley

St. Patrick’s Day, Snakes and Hemophilia

Snakes show up in Irish history for one important reason: legend has it that St. Patrick (who was not a legend but a real person) banished snakes from the island. It’s a myth but in keeping with the idea of snakes as evil.

Snakes do get a bad rap, starting with Genesis in the Bible—he’s the Devil in disguise, and encourages Adam and Eve to disobey God and eat fruit from the tree of wisdom, and we all know how that went. That theme is continued in Exodus, when Moses and his brother Aaron use their staffs in a competition with Pharaoh’s magicians. They each turn their staffs into snakes, and Aaron wins when his snake devours the others.

Staffs and snakes go together importantly in medical history—for healing. In fact, the use of snakes as a medical image dates back to 1400 BC. The Caduceus is a symbol of Hermes (Greek) or Mercury (Roman) in mythology. The Caduceus is a short staff entwined by two serpents, sometimes surmounted by wings. Mercury was the god of speed—that’s why our planet with the shortest (think fastest) orbit around the sun is named Mercury. It only takes the planet 88 days to orbit, compared to earth’s 365 days.

The Rod of Asclepius belongs to Aesculapius, the Greek god of healing. The American Medical Association started using the staff of Aesculapius as its symbol in 1910. The Royal Army Medical Corp, French Military Service, and other medical organizations had done the same. Even today organizations like the World Health Organization use the staff of Aesculapius in them. 

You may even find it or the Caduceus on your child’s medic alert bracelet. It’s a universal symbol of a medical condition. And the funny thing is, certain snake venom has been shown to clot blood in some cases!

Happy St. Patrick’s Day, even if you are not Irish, and make sure to wear a medical ID when celebrating—and every day.

A welcome visitor in my backyard
https://ashpublications.org/blood/article/9/12/1185/7359/The-Clotting-Action-of-Russell-Viper-Venom

An Irish Genealogy of Hemophilia—and a Mystery

With St. Patrick’s Day coming up, I thought it would be interesting to look at an Irish tale involving hemophilia. From our archivist and researcher from North Carolina, Richard Atwood: “Ghosts of the Missing (2020) is a cold case missing person mystery that accurately portrays hemophilia, then weaves it into the multigenerational story of missing girls and their ghosts. The author, Kathleen Donohoe, who serves on the board of Irish Americans Writers & Artists, lives in Brooklyn, New York.” From Amazon: In the vein of The Lovely Bones and The Little Friend, Ghosts of the Missing follows the mysterious disappearance of a twelve-year-old girl during a town parade and the reverberations of this tragedy throughout the town.

William Moye builds his foundry in the Hudson River Valley in what will become Culleton, New York. Irish-Catholic immigrants work in the foundry, which specializes in creating bells. The workers live in the shantytown called Cullytown. Helen Dunleavy, age 16 with green eyes, arrives in 1856 to work in the Moye household. She comes from Ballyineen (or Daughtertown) in Galway, Ireland. Dunleavy women are healers: they have the ability to cure. The women are also cursed: girls live, while the boys die in infancy. Helen brings to America the cure and the curse, or broken gene (later identified as factor VIII deficiency or hemophilia A). Her son dies, while her twin daughters, named Clara and Lucy, live. Clara does not have the gene. Lucy has it and passes it on.

Cassius Moye, the son of William, is born in 1843. He is captured during the Civil War, and develops tuberculosis. When he returns home, he writes The Lost Girl and Other Stories. His cousin Augustus runs the foundry. At his death in 1884, Cassius passes the Moye House to his publisher, who eventually sells it to Marjorie Moye Pearse, a descendant of Augustus, in 1946. The Moye House Writers’ Colony is established in 1953 to be located in the house where Cassius Moye was born. Culleton holds a Lost Girl celebration every October 27. Cecilia Burke, a descendant of Lucy Dunleavy, marries Daragh McCrohan and has two sons, Michan and Cathal. Both sons have hemophilia. Their factor VIII is a miracle treatment: it saved them before it killed them. “Factor VIII was a blood product manufactured as a powdered concentrate that caused blood to clot.” (p. 79). Michan, born in 1967, is spared HIV due to immunity or resistance, but acquires Hepatitis C. He becomes a poet and a professor at Gilbride College.

As a bachelor, Michan takes up residence in Moye House and publishes Lost Girls: An Anthology of Stories Written at Moye House Writers’ Colony. Cathal and his wife Lissa acquire HIV and die of AIDS, while their daughter Adair develops AIDS. Michan brings the orphan Adair, then 11, to Moye House in 1994. Rowan Kinnane is a fifth cousin of Adair McCrohan (Helen Dunleavy is four times great grandmother to both girls). Rowan, a descendant of Clara Dunleavy, befriends Adair, who is shunned at school for having AIDS. On October 28, 1995, Rowan, aged 12, disappears under suspicious circumstances. The police never solve her case.

Adair starts HAART treatment, an antiviral cocktail, in 1996. Adair experiences visions of Rowan. In 2010 while living in Brooklyn, she stops taking her medicines to be hospitalized with pneumonia. She returns to Moye House under the care of Michan, once again. Ciaran Riordan, a stepbrother of Rowan living in Ballyineen, Ireland, enrolls in the Moye House Writers’ Colony to research missing children, including Rowan. Both Adair and Ciaran investigate the disappearance of Rowan by eliminating suspects and finding clues associated with the rowan tree, or quicken tree, located next to the Rosary Chapel and a book of magic called A Charm for Lasting Love: Spells and Cures from Ireland, that includes a cure for the chronically ill.

Adair McCrohan explains hemophilia: “Culleton had known for a century that my family was cursed. Boys die. Girls live. At least this was how they put it before the disease was called by its proper name: hemophilia. From the Greek: haima = blood + philia = to love. Though ‘to love’ in this context is interpreted as “tendency to.” The blood doesn’t clot properly. Tendency to bleed. Women are carriers. Their sons get the disease but their daughters don’t.”

The Amazing Dr. Mütter: Father of US Plastic Surgery

“But what sustains the physician, in the stillness of night, in the chamber of pestilence, in the reeking hut of the sick beggar—in the cell of the maniac? A moral courage, which bids him die rather than desert his charge—a God, who tells him that “a faithful shepherd must give his life for the flock!” Thomas Dent Mütter

Dr. Thomas Dent Mütter

Last week I took a brief trip to Philadelphia, the birthplace of modern medicine in the United States. I am fascinated by the history of medicine—perhaps because I had a son with hemophilia in 1987, although I can recall desperately wanting to be a veterinarian as a child. Nowadays, I just read everything I can about medicine through the ages. For this trip in particular, I wanted to revisit The Mütter Museum, an odd and eclectic collection of anatomical and medical anomalies, kind of a Ripley’s Believe It or Not for medical students.

But you don’t have to be a medical student to learn a lot from this astounding collection.

And while it is Bleeding Disorders Awareness Month, and we are all sharing information about bleeding disorders in the US, I think it is good for us all to appreciate the humble roots of all medicine, and especially appreciate the remarkable legacy given to us by Thomas Mütter, who was born this week, March 9 in 1811 in Richmond, Virginia. Sadly, his mother, father and  younger brother died by the time he was seven. His maternal grandmother took him in, but then she died not long after. He was then raised by a wealthy friend of his father, who put him in a boarding school. Thomas, now lacking any family and suffering bouts of colic, decided to become a doctor. And what a doctor he would become.

In the 1800s, Paris was the epicenter of medical innovation, and Mütter would eventually study there, and witness poverty and suffering. Even still, life was harsh and brief then. Children were abandoned; more than sixteen thousand children were wards of the Hôpital des Enfants-Trouvés, and four thousand of those would never live to adulthood. He saw how open-minded the French were in their approach to medicine. He studied under legendary physicians, especially

Guillaume Dupuytren, of Paris’s largest hospital, the Hôtel-Dieu, the city’s, who altered the course of surgery. Dupuytren performed les opérations plastiques—plastic surgery. “Plastique” doesn’t mean plastic, but flexible. This surgery hoped to reconstruct injuries using the patient’s own body, such as tissue, skin, or bone, to heal.

At age 21, Mütter returned to Philadelphia, where there were (and still are!) two superb medical colleges—the University of Pennsylvania and Jefferson Medical College. In fact the University of Pennsylvania School of Medicine became the first and only medical school in the thirteen American colonies in fall of 1765. Surgery at this time was still crude. There was no anesthesia, and the best surgeons were usually the fastest. Mütter was ambidextrousness, and could do twice the work in half the time, it was said. Public sanitation was nonexistent, and Philly, like most big cities, was hit hard with epidemics.  In 1793, yellow fever struck; in 1832, a cholera epidemic hit. Without electricity, surgeries were performed in natural light or by candlelight.

Mütter was such a notable surgeon, so talented, that he was awarded the chair of surgery at Jefferson College. He was their youngest and least-tested professor.

He appeared to be beloved by his students; his lectures were fascinating. At a time when lecturing was very one-sided—students listening and professor talking—Mütter was first US professor to introduce a more informal style of teaching, asking questions and engaging in dialogue with his students. Mütter’s reputation was also enhanced by his habit of bringing specimens to class—preserved body parts, often in jars. These would form the basis of his museum.

He had other firsts: introducing a new type of plastic surgery that is still today used, called the Mütter flap. Keeping patients overnight in recovery beds, instead of being sent home the day of surgery in horse-drawn, unsanitary carriages with wooden wheels. Suspecting that physicians introduced germs to patients during their physical exams as they did not wash in between patients—sepsis, which would not be confirmed for years later. Becoming the first surgeon in Philadelphia to administer anesthesia for surgery, even though his peers did not trust the newfangled chemical. He struggled for anesthesia to be adopted by the medical community to end unnecessary human suffering—proving himself to be a hero to patients.

And with all his accomplishments, it was perhaps his compassion and tenderness towards his patients that made him different from everyone else. From students to peers to superiors, everyone noted Mütter’s gentle manner, listening skills and time spent comforting the suffering. Perhaps because he himself had suffered so much in life.

“’Brilliant as Dr. Mütter was in his didactic teachings,’ one of his students later wrote, ‘he surpassed himself in the clinical arena.’ Mütter’s methodical nature and utter focus before, during, and after the surgery was unprecedented—as was the amount of empathy and kindness he displayed for those under his care. Furthermore, Mütter was known for putting ‘considerable emphasis’ on the care and attention he paid to patients prior to performing the operation. He would not only develop a bond with the patient via gentle, consistent communication, but would also physically as well as mentally prepare them.

“Because of this care, Mütter’s work and reputation rose above that of his contemporaries. He even treated a man who suffered horribly from elephantiasis and, immediately afterward, took up a collection for the man, reminding the students that compassion for someone like this does not stop at the operating room door.”•

His legacy is vast. For example, he inspired people like Edward Robinson Squibb, who was inspired to find a way to standardize ether, and provide doctors and surgeons with standardized chemicals for their work. His advocacy later led to the creation of the US FDA.

And then there is the Mütter Museum. Mütter had seen jars of fetuses and deformities in anatomy in Paris, labeled “MONSTER.” He brought a feeling of humanity to these unfortunates. By keeping them on display, he taught his students compassion for those who suffer. At first Jefferson College refused to create such a museum, but Mütter knew his time on earth was short, and pushed. Finally, in December of 1858, an agreement approving the Mütter Museum was, at long last, legally recognized. Dr. Thomas Dent Mütter died a mere three months later, at age 47.

Try to visit the Museum if you can; Philadelphia is a city filled with history, of our nation—and our medicine.

“This world is no place of rest. It is no place of rest, I repeat, but for effort. Steady, continuous undeviating effort. Our work should never be done, and it is the daydream of ignorance to look forward to that as a happy time, when we shall wish for nothing more, and have nothing more to accomplish.” Thomas Dent Mütter

  • Excerpted from Dr. Mutter’s Marvels: A True Tale of Intrigue and  Innovation at the Dawn of Modern Medicine by Cristin O’Keefe Aptowicz

U.S. Presidents and Hemophilia

Monday, February 19, is President’s Day, which used to be called Washington’s Birthday, in honor of our first president. It got me thinking about whether U.S. presidents have any connection to hemophilia. For the sake of history—our hemophilia history—you should know that they do.

Thanks to the advocacy efforts of the national hemophilia community, President Ronald Regan, our 40th president, issued on February 21, 1986, a proclamation declaring March “Hemophilia Awareness Month.” This was, of course, at the height of the national blood infection scandal—it took Regan’s actor friend Rock Hudson to come out as homosexual and share publicly that he had HIV for the Regan Administration to finally pay attention to what was happening. That is a whole story in itself (Read And the Band Played On to learn the history). It was a dark time, still remembered by many of us.

But the proclamation at least gave our community national, positive recognition.

Now, we celebrate advances made in treatment and technology. And as our community becomes more inclusive, it is called Bleeding Disorders Awareness Month. Read the original proclamation below…

February 21, 1986

By the President of the United States of America

A Proclamation

Hemophilia is a genetic disease in which the blood will not clot properly because a specific clotting factor is missing or defective. It is almost exclusively a male disease, affecting one of every four thousand live male births, regardless of race, nationality, or family economic status.

Significant advances have been made in the diagnosis and treatment of hemophilia. Methods of early diagnosis provide the means to institute preventive measures at an early age. Modern blood fractionation technology provides a moderately priced, readily available supply of the needed clotting component for those who must have regular infusions. Still, hemophiliacs face a life of unpredictable medical complications that may result in disability or death.

Proper medical treatment for the hemophiliac, and public awareness and acceptance of this medical condition, will allow the individual to live a normal, productive, and independent life and will dispel many of the common misconceptions of the disease.

To stimulate public awareness about hemophilia and to encourage the development of improved techniques of diagnosis and treatment, the Congress, by Senate Joint Resolution 150, has designated the month of March 1986 as “National Hemophilia Month” and authorized and requested the President to issue a proclamation in observance of this period.

Now, Therefore, I, Ronald Reagan, President of the United States of America, do hereby proclaim the month of March 1986 as National Hemophilia Month. I invite the Governors of each State, the Commonwealth of Puerto Rico, the officials of other areas subject to the jurisdiction of the United States, and the American people to join me in reaffirming our commitment to finding better ways of helping those afflicted with hemophilia.

In Witness Whereof, I have hereunto set my hand this twenty-first day of February, in the year of our Lord nineteen hundred and eighty-six, and of the Independence of the United States of America the two hundred and tenth.

Ronald Reagan

Alternative Pain Relief

Pain is highly personal. No two people experience the same feeling of pain, even when it’s the same injury, like a muscle bleed, or experience, like childbirth. A joint bleed may feel tingling to one, stabbing to another, or throbbing to someone else. A man with hemophilia A said, “Pain is pretty deeply personal. I personally have never been able to figure out what to say when a nurse asks me to describe my pain.”

            But it’s especially personal when trying to describe the level of pain. Doctors often ask patients to rate their pain on a scale of 1 to 10. But what is a 1? What is a 10? A level 8 to one person might be a level 3 to another. Ed, who has hemophilia A, notes, “The HTC [hemophilia treatment center] will understand that most of us older guys have a base pain level that stays steady at a 5 or 6 every day. We’ve gotten used to that level of pain and this is our ‘normal.’ What’s difficult is when you go to an ER and try to relay that same information.” This is critical when people with bleeding disorders try to explain their level of pain to their doctor. Not appreciating or understanding how much pain a person is feeling may lead to an inefficient treatment for that pain.

TENS therapy

            Bonnie interprets her pain at lower levels when compared to people without a bleeding disorder. “I feel like what would be painful to someone else is just the norm for me. And I don’t find it painful because I’ve learned to live with it.”

            Because pain is so personal, medication may not be the first—or the only—option for chronic pain. Instead, both patient and physician can consider different types of complementary and alternative medicine (CAM) to learn how to handle chronic pain. And like pain, CAM can be highly personalized as well.

What Is CAM?

CAM is any adjunct (additional) therapy, like massage, used along with conventional medicine. It’s an important part of a multimodal or multidisciplinary approach to pain management. It’s also important in integrative medicine, which focuses on the whole person and makes use of all appropriate therapeutic approaches, healthcare professionals, and disciplines to achieve optimal health and healing. Here are some of the most common CAM therapies:

            Relaxation Therapies. Relaxation teaches you to relieve tense muscles, reduce anxiety, and alter your mental state. Mindfulness meditation helps you focus attention on a specific object or your breathing patterns to induce relaxation. Guided imagery is a conscious meditation technique of relaxation followed by visualization of a soothing mental image, like walking on a beach at sunset.

            Biofeedback Training. You can learn how to recognize and change your biological reactions to stress and pain by using electronic equipment to monitor your physical responses: brain activity, blood pressure, muscle tension, and heart rate.

            Behavior Modification. Some people with severe chronic pain may become anxious, depressed, homebound, dependent, or bedridden. Behavior modification helps you create a step-by-step approach to confronting challenges by changing your behavior and shifting your attitude. Matt Barkdull, a man with hemophilia B who is also a licensed mental health specialist, says, “Behavior modification and stress management are my go-to interventions. I resist the urge to curse my bad luck, attack my self-identity, or become bitter (for that which we harbor is that which we attract). I believe pain is there to teach me a lesson, to remind me to appreciate better days ahead. When I meditate upon these things, I become more grateful for the important things in my life, and make better decisions. These interventions seem to work best when pain is dull but constant and for bleeds that are relatively minor but have caused some mobility problems that will require a little time to heal. Spiking and blinding pain (deep muscle bleeds from injury) often requires me to reach out and share my struggles, perhaps take a pain pill or two, and seek some relief. It’s hard to be mindful while battling the sting of acute pain. However, I find if I deliberately engage in deep-breathing exercises and stay connected while avoiding allowing my mind to wander and unhinging from false perceptions, the pain is much better controlled.”

            Stress Management Training. If your pain level is high, your stress levels probably are, too. This training helps you maintain a routine schedule for activity, rest, and medication. It incorporates exercise or physical therapy into your daily routine, and trains you to keep a positive outlook.

            Hypnotherapy. Therapeutic or medical hypnosis directs your focus inward to help you relax and reduce pain and anxiety. You can learn self-hypnosis from a trained hypnotherapist.

            Counseling. Individual, family, or group counseling with a professional trained in pain management can provide emotional support and guidance. Tina, mother of two young children with hemophilia A, notes that anxiety is a type of pain: “Most of my boys’ pain is anxiety-related. It causes discomfort. I feel my children are more anxious than non-hemophilic kids because they associate injury with the added step of factor.” George adds, “Speaking with a mental health professional and learning meditation helped me the most. I can’t tell you how at peace I became when my mind accepted the fact that pain is part of my life and I can turn it into power and motivation to help others.”

            Acupuncture. Many patients report pain relief from this ancient Chinese technique of inserting and manipulating thin needles into specific points on the body known to control pain pathways.

            Dozens of other therapies, including acupressure, massage, and chiropractic manipulation, may help control pain. Transcutaneous electrical nerve stimulators (TENS) deliver electrical impulses to interfere with pain transmission. Ultrasound therapy warms joints internally to provide pain relief, and laser treatments may provide relief in a similar way.

            A good management plan for chronic pain must be personalized. It should use a multimodal approach, which addresses the psychological component of chronic pain by treating depression and reducing anxiety and stress. A multimodal approach includes adjuvant therapies (antidepressants and anticonvulsants); an exercise and/or physical therapy component; and some form of CAM, which allows the person to manage moderate to severe chronic pain with the lowest possible dose of painkillers.

            Here’s how Max, a person with hemophilia A, sums up personalized pain: “I’ve had to learn to understand my pain in ways that were perhaps discouraged at an earlier age.  Pain is a friend; it’s part of me. I’m learning from it every day and learning to live with it makes it less of a burden.”

Acupuncture is safe for people on prophylaxis. If you’re considering acupuncture, first talk to your hematologist or the staff at your HTC.

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