Wine and hemophilia: what’s the connection?

“A bottle of wine contains more philosophy than all the books in the world.” Attributed to Louis Pasteur

Was wine ever considered historically as a treatment for hemophilia?

Richard Atwood, our contributing writer from North Carolina, offers this week’s blog about the history of wine as a medicine, by summarizing a book he read.

Salvatore P. Lucia’s 1963 book, A History of Wine as Therapy, purports that wine is perhaps the oldest of all medicines, dating back over 40 centuries of written record. Wine is also used as a menstruum for other therapeutic substances. Wine contains alcohol, aldehydes, ketones, esters, acids, carbon dioxides, nitrogenous compounds, pigments, tannins, sugars, pectins, glycerols, vitamins, and inorganic compounds. As a food, wine provides fluids, calories, minerals, vitamins, and proteins. As a medicine, wine acts an appetite stimulant, stomachic, tonic, tranquilizer, anesthetic, astringent, antiseptic, vasodilator, diaphoretic, diuretic, and antibacterial agent! Whew!

Wine was used in the healing arts in Egypt, India, China, Greece, Rome, Byzantine, Syria, Persia, the Dark Ages, and modern times. The therapeutic use of wine is documented in Egyptian medical papyri, the Bible, the Talmud, the Koran, Hindu vedas, Chinese medical treatises, plus ancient and modern formularies, dispensaries, and pharmacopoeias. Wine is prescribed as a pharmaceutical necessity. Among the prominent physicians promoting wine as therapy are Hippocrates, Socrates, Plato, Aristotle, Celsus, Homer, Pliny, Dioscorides, St. Luke the Evangelist, Galen, Avicenna, Maimonides, Arnald of Villanova, and Ambroise Pare. So maybe there is some philosophy to this!

Research scientists investigated the effects of the polyphenols and tannins found in grapes. These substances are thought to promote the resistance of the capillaries to hemorrhage. This would “confirm the empirical observations of the Spanish court physician who prescribed a diet of grapes and wine for the Prince of Asturias, the first recorded haemophiliac member of the royal family of Spain.” (pp. 197-198). The reference is an undated, unpublished manuscript by A.J. Lorenz.

Richard comments that there is only one reference to hemophilia in the text, but seven references to anemia. The absence of much hematological research on wine is disappointing. The use of wine as a treatment for hemophilia, especially when the patient with hemophilia is Spanish royalty, has seemingly never been replicated.

For the use of wine to treat hemophilia, Lucia omitted the reference to John Cochrane, MD, surgeon in Edinburgh, Scotland, who published in Lancet (ii: 147-150) the 1841 article titled “ On the haemorrhagic diathesis.” Cochrane discounted the effectiveness of the internal use port wine as an effective treatment of hemophilia because the wine increases the general force for circulation, causing the congested vessels to rupture. Ouch.

Maybe wine is best left to the parents or caregivers, to relax them from the aftermath of a hospital visit or surgery in their loved one with hemophilia?

Cheers!

Hiking for Hemophilia

Yesterday was a beautiful, sunny, crisp fall day in New England, and perfect for a hike. Hiking is even better when you can hike to help!

The Hemophilia Alliance of Maine (HAM) hosted its fourth annual Hike4HAM, at Camden Hills State Park, where we would hike up Mt. Battie. A one-hour hike up offered spectacular views of Penobscot Bay, a way to earn a great lunch, and enjoy the company of our “family,” the hemophilia community.

This was really special for me. For years I’ve been promising the dedicated staff at HAM I’d try to make their walk, or hike, or annual meeting. Something always came up that interfered with that plan. But Saturday, September 15… all clear to go! Doug and I drove the three hours to Camden Hills, enjoying the New England scenery and quaint towns. Maine is an absolutely beautiful state, “The Way Life Should Be,” as its slogan says.

I didn’t think I knew any of the families. One of the reasons HAM was founded is that it’s so difficult for Maine families to come to the New England regional events. Although New England is significant in size, a combination of six states, we really do have our own cultures. Massachusetts is different than Maine, which is different than Vermont or even New Hampshire. I was looking forward to meeting new families and maybe connecting with some who might be on our mailing list.

 

The Greene Team!

Families were grouped in teams, usually with a name of a family member with a bleeding disorder, like “Team Thomas.” Industry reps were there with tables of product info and giveaways, and donuts! It was fun to connect with known friends like Sandy (Octapharma), Tommy (Novo Nordisk), Ron (CSL Behring), Lisa (Bioverativ) and Victoria (Aptevo). And to see Jill Packard, president and founder of HAM. As we started our hike in the chilly air, it progressively got warmer as we moved, and I fell in to chatting with an adorable 10-year-old named Emery.

Emery

A long time ago I seemed to have more time and focus to chat with families, and got to know so many through these past 30 years. As we hiked, I loved asking Emery all about his hemophilia: he has hemophilia A, he told me–“…that’s factor VIII.” And he self-infuses–impressive! His little brother Hamilton has hemophilia too. Watching Emery I was impressed at his stamina, and absolutely no joint damage. You would never know this child has hemophilia. He jumped off a boulder, landed hard and kept hiking!

Families hiked in teams, and through the woods came moms, dads, uncles, aunts, friends, grandparents, lots of children and even dogs!

An hour later, we were rewarded with a view of clouds… we were above the cloud and fog line, which obscured the bay but still made for a worthwhile climb. As did the fundraising: HAM raised about $20,000 on this hike, which we were proud to contribute to–I even got a medal for being one of the top donors!

After the hike we had hamburgers, corn on the cob, potato salad and lemonade. I sat with folks I didn’t think I knew, but as we shared names, they knew me and shared stories of how my book Raising a Child with Hemophilia had helped them, especially back in the days long before HAM was founded. One mom told me RCH and our newsletter PEN were the only connections she had with the hemophilia community. That was so sweet to hear! And with HAM, she is now well connected.

Congratulations to Jill and team for creating HAM, for holding the hike, and for all the participants, particularly the industry reps who give up so many weekends to participate in our local events. I hope to hike next year too!

See Complete Gallery of Photos Here

Laurie Kelley with Lisa Schmitt and family

 

He Planted Kindness: Kyle Callahan

 A tree is known by its fruit; a man by his deeds. A good deed is never lost; he who sows courtesy reaps friendship, and he who plants kindness gathers love.  —St. Basil

I’m in Nashville, waiting to board my flight home, leaving behind many hemophilia community members who came to pay tribute to a fallen giant, Kyle Callahan. Kyle, a 52-year-old with hemophilia, passed away suddenly on August 26. For a man who had survived so much in his life, and engaged in outrageous adventures and high-speed fun, it was stunning news.

Nashville baked in 90° weather when I arrived yesterday afternoon; I hurried over to Union Station, a magnificent building and scene of Kyle and Diane’s wedding 24 years earlier. It was healing to see so many people in our community that I’ve known for decades and admire: Craig Mears and wife Kelly, John Jarratt, Dianne (Martz) Griffith (Kyle’s mother), Louise Hardaway, who helped Dianne set up her original business in 1980 (and is mother-in-law of Pat “Big Dog” Torrey), and more. Our community runs deep in memories, connections and affection.

Diane and Kyle Callahan

Inside the big room where we gathered was a head table filled with memorabilia: A Harley Davidson sign, a signed football, a photo of his beloved Doberman, a collage of the many adventures he and his wife Diane shared.

Kyle was born in South Dakota, but always revealed a bit of a southern accent, after living in Nashville most of his life. I met Kyle in 1992, after first meeting Dianne, his mother, who had just started Hemophilia Health Services, a specialty pharmacy that prided itself on the “human factor.” More than just a slogan, HHS was the booth at NHF meetings that had soft colors, music, chairs and warm representatives. Dianne created a new way of providing factor to customers, with a high-touch, personable approach. My first meeting with Kyle is imbedded in my mind: a tall, good-looking 26-year-old who looked years younger, with strawberry-blond hair, freckles and a dazzling smile. About 7 years later, Dianne retired and Kyle was nominated by the board to become president. I wondered how this “young man” could possibly take on this responsibility?

He assumed responsibility and would later rocket HHS to become the largest hemophilia specialty pharmacy in America. From revenues of $30 million to over $1 billion.

I watched Kyle grow, and he became a friend, a colleague, mentor and supporter of my work. I needed an article reviewed for accuracy; Kyle agreed to do it. I needed a book reviewed—Kyle again agreed! I needed to interview a captain of industry about changing insurance—Kyle again. I wanted to set up a factor donation program to stop the waste of unwanted factor—Kyle invited me to Nashville to learn how their operations ran. That tour became the basis of how we constructed Project SHARE, so we were in compliance. And that came in handy when one morning at work I looked up, and an FDA agent named Jason was staring down at me, badge in hand, because he heard we were shipping “drugs.” We sailed through our first inspection with flying colors.

HHS collected factor that patients returned, and donated them to us. Kyle helped fund the program, which continues today, and which has given over $130 million worth of factor to developing countries.

When I wanted to start a child sponsorship program, Save One Life, Kyle again agreed to help support us. And he and Diane sponsor 17 children with hemophilia in developing countries.

Laurie Kelley with Kyle’s mother, Dianne Griffith, founder of Hemophilia Health Services

But above and beyond all this, the most important, most impressive things about Kyle were not so quantifiable. That charming man with the dazzling smile I met in 1992 never once wavered in civility, humility, and kindness. And he endured much; at one NHF meeting, I barely recognized him. I’m afraid I stared when I saw him; he was so gaunt from his illness. But still, that smile. That charm. He always was gentle. He always was available. He seemed to carry no baggage; he was present, in the moment, focused on whoever he was with. He laughed easily and smiled perpetually. He was the real deal. That’s called integrity.

Kyle and I connected on helping others less fortunate, but also on the lighter side of things. We were both adventurers, and I loved reading about his adventures and he liked knowing mine. I joke that he flew planes; I jump out of them. He drove race cars; I tried cooking. We both loved traveling to foreign places, the more exotic the better! I always tried to send him a birthday card each June, except for last year when I confessed I was climbing a volcano in the Congo; he gave me a pass!

After he retired for a time, before he started a new venture, he and Diane took a few years to travel the world. I have Christmas cards of them in Egypt, sitting on camels; holding a koala bear in Australia.

When I learned he and Diane were going to Antarctica, I was so excited for them and envious—it’s a place I have always wanted to visit. I’m a voracious reader about polar exploration. Following an NHF meeting, waiting at the airport, I shared my knowledge of polar exploration and especially the Shackleton story. Kyle assured me they were going to South Georgia island to see his grave. It’s a hunk of rock that contained an old whaling station. Would it be possible to send me a postcard from there (in case I never get there myself)?

Not long after I received a postcard of Ernest Shackleton’s gravesite from Kyle and Diane. That is kindness itself.

I  have kept that postcard on my bookcase, next to all my travel and adventure books, to remind me to go there someday. Someday…

The postcard seems to be a calling card now, to get with it, and live life like Kyle. Our days are numbered, and no one knows when we will depart. Kyle flew planes, scuba dived, drove race cars, was a pilot, drove a Harley, commanded a beautiful yacht. And through all of it, he never lost his gentle grace, his smile, his zen-like composure, his kindness, his humility. He never said a bad word about anyone. And no one, not even his competitors, ever said a bad word about him—an amazing feat in the specialty pharmacy business. Everyone who spoke yesterday said the same thing: he was perhaps the kindest person you could ever meet.

Kyle’s message to me seems to be: 1) Live each day to the fullest, 2) chase your dreams; they can come true! and 3) be kind. Always. To everyone.

Good-bye Kyle, and thank you for leaving the world a better place. More adventures await you, and we will try to catch up with you one day!

Diane Callahan has asked that donations in Kyle’s name be made to www.SaveOneLife.net, or to the Tennessee Bleeding Disorder Foundation.

 

 

 

How does your school-age child understand hemophilia?

Published in August 2018 PEN

With the start of the school year comes new teachers, nurses, and caretakers for your child. You may be explaining to many adults what hemophilia is and how they should properly respond if your child has an issue. But, how do you go about teaching your child about their hemophilia? In this excerpt from our latest issue of PEN, we delve into how children understand their bleeding disorders and what you can do to help present information  for them to digest. 

Teaching Your School-Age Child About Hemophilia 

One of the biggest challenges we have as parents of children with hemophilia is teaching our children about their disorder. We often use words like hematoma, factor, and deficiency; and concepts like prophy, coagulation, and heredity. But children understand these words and concepts very differently than adults do.

If you don’t know how your child’s mind works at various stages of his development, then teaching him about hemophilia becomes hit-or-miss. But when you know how he thinks, you can tailor information in a way that he can easily understand. So to teach your child about hemophilia, you need to know how he processes his world in general, and hemophilia concepts in particular.

 

The School-Age Child’s Thinking Tools

Between ages 7 and 11, the school-age child is in a fascinating stage of cognitive development. “Cognitive” refers to how he thinks, how he processes incoming information about his world—basically, his ability to think logically. Just as he has a skeletal structure that develops as he grows, he also has a mental structure that develops as he matures, filtering information in a way he can grasp.

Your child’s mental structure is characterized by five major thinking tools that are constantly evolving:

Causal thinking: Figuring out when something causes something else, using a step-by-step process. A preschooler doesn’t typically think step-by-step.

Internalized thinking: Moving from understanding his world mainly through his senses—where things happen outside him—to realizing that things can happen inside him.

Gradient thinking: Knowing that the world isn’t just polar opposites, like good guys and bad guys. There are now shades of gray, degrees of intensity. A good guy might do something bad. Your child can also distinguish parts from the whole.

Empathic thinking: Starting to see the world from another’s point of view.

Time: Understanding that he doesn’t exist just in the present, but that he has a past and a future.

For understanding hemophilia, the most important of these five thinking tools may be causal thinking. Your child can now try to figure out how one thing causes another. Like…What causes bleeding? A blood clot? What is genetic transmission? It’s hard to explain these concepts when your child doesn’t understand causality. These are more sophisticated thinking tools than he had as a preschooler, yet a school-age child, ages 7 to 11, is most comfortable using his new thinking tools on things and places he knows best—the tangible, visible world. So let’s see how he uses these thinking tools on various topics in hemophilia, starting with blood.

How He Understands Blood

Unlike a preschooler, your school-age child understands the concept of the whole and its parts. So you can explain blood in terms of what it’s made of. Children between ages 7 and 9 believe that blood is a red liquid, but also that it’s composed of “stuff—water, food and energy.” Children between ages 9 and 11 tend to describe blood in more abstract terms. “It’s cells. Little roundish stuff. They’re red and blue.” A child develops from concrete to more abstract thinking, so this is perfectly acceptable!

Now you can introduce the idea that blood has components: white blood cells, red blood cells, and platelets. While preschoolers focus on things outside the body, mainly what they can see, hear, and feel, a school-age child realizes there are things inside him that he can’t see. So he’s ready to learn about simple blood components, especially those related to his hemophilia.

How He Understands Hemophilia

Because he understands a whole and its parts, your child can now categorize things. A preschooler might describe hemophilia as “blood,” or “something I have,” but a school-age child can classify hemophilia as a “blood disorder,” or “when blood doesn’t stop bleeding.”

He also progresses from describing hemophilia as his own specific injury (“It’s when I get a hurt knee”) to seeing it as a condition (“It’s when someone gets hurt and bleeds a lot”). This is the empathic thinking tool: he knows he is not the only one to have hemophilia. He now says that hemophilia is when “boys with hemophilia have to go to the hospital sometimes.” Compare this to the preschooler reply, “When I have to go to the hospital.”

Your child also has matured from an external to a more internal focus. A preschooler might say, “Hemophilia is bruises,” but a school-age child will say, “My blood doesn’t work right.” What is it that doesn’t work right? Well, he understands the concept of a whole and its parts, and he’s ready to know that blood is composed of parts. So he can deduce that hemophilia means “something’s missing” in his blood. Some children say that they have “lost” something, or that their blood is “too thin.” These answers reflect the “something’s missing” idea. For example, “It’s when you’re missing some factors that help to make it so if you slam your knee against something it doesn’t swell up as much. You’ll have to replace the factor.”

Misconceptions and medical inaccuracies abound as your school-age child struggles to understand hemophilia. “It’s a blood disease. You lose part of your blood and you need to get more blood.” At this stage, what’s important is not so much that his answers are right or wrong, but how he arrives at his interesting conclusions.

So teach your child that hemophilia is a “blood disorder.” Teach him that blood is made up of parts, and that he is “missing” a part. There’s no need to get too specific at first, for example by mentioning factor and proteins; just stick to general concepts and ideas. To help him visualize, use a concrete example, like the falling dominoes. Remember that a school-age child is increasingly able to understand more abstract terms, but he needs the help of concrete examples.

How He Understand Genetics

Learning about heredity is a great way to exercise the “missing step” concept in a step-by-step sequence. To a preschooler, hemophilia is just something he was born with. To a school-age child, something had to happen to cause hemophilia.

What is that something? His parents are usually the missing step. Your child possesses the thinking tool of time, so he may realize that hemophilia could have started in his family many years ago, even centuries ago.

But how exactly does hemophilia get from one person to another? Most school-age children name a causal agent—the thing or event that caused hemophilia to happen. This can be a parent, blood, a chromosome, sperm, or even “vibes,” as one boy phrased it.

Understanding often differs among younger children (ages 7–9) and older children (ages 10–11). When asked how they got hemophilia, younger children may simply reply, “Mom” or “Mom and Dad.” Some children name blood. “It streams through your family, through their blood. I got it when I was two or three. My uncle gave it to me.” This child is medically incorrect, but he’s trying to sort it out logically: a family member had hemophilia, I have hemophilia, and…maybe my uncle gave it to me?

School-age children may also understand or accept some basic heredity rules, such as “mothers are carriers.” This makes sense to them, because a “carrier” is a causal agent.

From ages 9 through 11, a child’s answers and thought processes become more sophisticated. The causal agent may be chromosomes, which only a few children can discuss at this stage. Remember that school-age children are still very concrete—more comfortable with things that they see, hear, feel, and smell. Chromosomes are abstract. Some children identify an “X thing” as the causal agent, but don’t understand the idea in purely scientific terms. To them, X and Y are not parts of the cells. They’re more like “germs” that other family members “catch.” One boy explained, “Mom’s a carrier. She has two little things inside her, little Xs. They’re like little eggs. She has a good X and a bad little X in her. My brother got the good carrier and I got the bad carrier.”

Ask your child, “Where did your hemophilia come from?” and let him figure it out on his own; don’t judge his answer at first or try to answer for him. You can work on the details later, supplying more accurate information once you’ve listened to his explanation and understood his thinking.

 

To read more on this topic download our August 2018 PEN today at

https://www.kelleycom.com/product-category/newsletters/pen/

Begging Your Pardon, Ma’am

This past week I received two messages in response to our August issue of PEN, from two different people, both concerned about our predominant use of the word “he” whenever we wrote about hemophilia in the issue. Was it an oversight? Aren’t we aware of the growing movement to get women recognized not just as carriers, but as people with hemophilia? It matters: women as just carriers with symptoms don’t get the same treatment or attention as males with hemophilia. And this can be dangerous.

We certainly recognize this and wrote a full feature article about this in the November 2016 issue of PEN. And just a year ago, this great article below by Paul Clement. On page two of each issue we always state why we predominantly use the word “he”: because mostly it’s men who currently make up the majority of cases of hemophilia, but also, as writers and editors, it’s awkward to write he/she, or even to use “they.” However, back to the editorial board on this one! We will convene as a team, and decide for each article how to include the words she, girl, her more often. We promise. We are grateful to our readers for alerting us to this, and all we can say is, please accept our apologies. We will work to include women as patients, not just parents, more often in our publications.

 

And the Survey Says . . .  Carriers, Get Tested!

Paul Clement

Women have hemophilia too! Although much progress has been made over the past two decades in getting this message out, public awareness of bleeding disorders among women is dismal. Even women who are known carriers of the gene for hemophilia often don’t realize that they can have hemophilia and be at risk of bleeding. Even carriers confirmed to have bleeding problems, and diagnosed as “symptomatic carriers,” have run into roadblocks in accessing proper care.

Why are so many women undiagnosed?

The main reason is that they don’t suspect they have a bleeding disorder and don’t seek medical treatment. In spring 2010, a national study surveyed 1,243 women from the general public, aged 18 to 25, to assess their knowledge, attitudes, health behaviors, and menstrual experiences.1 Many questions were designed to determine if women knew the difference between “normal” and “abnormal” bleeding patterns. The results were disheartening. Most knew that a bleeding disorder is a condition in which bleeding takes a long time to stop (77%), or blood does not clot (66%). But the women surveyed didn’t know much about bleeding disorders, and only a few could identify risk factors for a woman with a bleeding disorder: periods lasting eight days or longer; bleeding through a pad or tampon in an hour or less; feeling a sense of flooding or gushing. Of the women surveyed who were identified as having one or more of these risk factors, only 20% had sought medical attention, and only 2% had been diagnosed with a bleeding disorder. Contrast this with studies showing that on average, 13% of women seeking medical treatment for menorrhagia (heavy periods) have von Willebrand disease (VWD)!2

Bottom line: Most women don’t seek medical treatment for menorrhagia, and if they do, few are correctly diagnosed with a bleeding disorder. To increase awareness, National Hemophilia Foundation (NHF), Hemophilia Federation of America (HFA), and other advocacy organizations have launched multiple programs for women with bleeding disorders. These organizations are now doing a great job providing resources for diagnosed women. But it’s obvious that we must do a better job, to reach more women in the general population—to educate them about the risk factors and encourage them to seek medical treatment.

Lack of physician awareness

What happens when women do seek medical treatment? We’ve made some headway, as results from two different surveys show. A 2002 survey of 376 members of Georgia Chapter of the American College of Obstetricians and Gynecologists wanted to understand methods of diagnosing and treating menorrhagia, and to determine physicians’ experiences and perceptions about bleeding disorders, particularly VWD.3 The results were enlightening—and

shocking. Only 3% of responding physicians considered VWD a likely cause of menorrhagia in women aged 15 to 44. When asked how many women with menorrhagia might have an

inherited bleeding disorder, physicians’ average response was “less than 1%.” Most shocking: after practicing an average of 20 years, 42% of responding physicians reported never

having seen a woman with menorrhagia who had a bleeding disorder. But statistically, each physician annually saw several hundred patients with menorrhagia who had VWD!  Not surprisingly, the survey showed that gynecologists rarely (3%) refer a woman with unexplained menorrhagia to another specialist.

Fast forward a decade. In 2012, a similar survey of 503 ob-gyns had more positive results: nearly 39% of obstetricians and 77% of gynecologists were likely to consider VWD or another bleeding disorder as a cause for menorrhagia.4 And over 80% who had seen patients with menorrhagia attributed the problem to a bleeding disorder. Perhaps most important, instead of referring patients to specialists only rarely, most physicians referred patients with menorrhagia to other healthcare providers, nearly 45% to hematology.

Although it’s hard to compare surveys of different physicians a decade apart, the change in demographics of the patient population at hemophilia treatment centers (HTCs) also reflects an increased awareness in the medical community of bleeding disorders in women. Between 1990 and 2010, the HTC population grew 90% from 17,177 to 32,612—and most of this increase was due to additional VWD patients.5 This increase is expected to continue; unfortunately, these numbers only scratch the surface of the estimated 3.2 million people in the US with VWD, half of them women.6

Hemophilia carriers with low factor levels

The normal range of factor VIII and IX is between 50% and 150%, with most people being close to 100%. Factor VIII levels often vary, and may more than double due to the effects of hormones and other variables, such as stress or pregnancy. Factor IX levels

normally remain fairly stable. Being a carrier for hemophilia puts

a woman at risk of bleeding because of low factor levels. Carriers usually have factor levels between 30% and 70%, with most around 60%. But factor levels in carriers can vary widely, with some in the high-normal range and others below 10% (in extremely rare cases, below 1%). Research shows that even women with mildly low factor levels—40% to 60%—are at risk of bleeding.7 They may experience not only menorrhagia, but bleeding after tonsillectomy, tooth extractions, surgery, or trauma from accidents; and prolonged bleeding from minor cuts or joint bleeds. This isn’t well known among many carriers, so they may not seek treatment.

Every bleeding disorder advocacy organization offers educational materials on this topic, and it’s probably a safe guess that every chapter newsletter has published multiple articles on the risk of bleeding in carriers. Yet for a variety of reasons, the message hasn’t been received by everyone affected.

But simply seeking medical treatment for excessive bleeding may not be enough—you may have to advocate for yourself. Carriers with bleeding problems are often diagnosed as “symptomatic carriers,” and a course of treatment is recommended. Everything should be okay for these women, right? Not necessarily. The word “carrier” often conveys the wrong meaning. In decades past, it meant you had the gene for a disorder, but you yourself didn’t show symptoms of the disorder. Many physicians who are not bleeding disorder specialists still have this definition in mind, but we now know that carriers can indeed have the genetic disorder. We don’t know for sure how many carriers have excessive bleeding, but a common estimate is that about one-third of carriers have factor levels below 50%, placing them at risk. These women have a factor deficiency and mild hemophilia. They have often been diagnosed as symptomatic carriers because some doctors resist using the word hemophilia based on the simplistic notion that “only males can have hemophilia.”

The symptomatic carrier diagnosis must be laid to rest. Not only is it misleading, but it often prevents women from getting the treatment they need. And insurance companies increasingly use a literal definition of “carrier” to deny coverage for treatment, arguing that symptomatic carriers don’t actually have the disorder. If your factor level is lower than 50%, request a diagnosis of mild (6% to 49%) or moderate (2% to 5%) hemophilia!

Knowing your factor level is essential. To rule out low levels, all women who are carriers should have their factor level checked (and if you are a carrier for hemophilia A, checked at least twice). If your levels are below the normal range, request a diagnosis of hemophilia. And get the word out: talk to your peers and let them know that most carriers are at risk of excessive bleeding.

 

  1. Patricia A. Rhynders et al., “Providing Young Women with Credible Health Information about Bleeding Disorders,” American Journal of Preventive Medicine 47, no. 5 (2014): 674–80. 2. M. Shankar et al., “Von Willebrand Disease in Women with Menorrhagia: A Systematic Review,” BJOG 111 (2004): 734–40. 3. A. Dilley et al., “A Survey of Gynecologists Concerning Menorrhagia: Perceptions of Bleeding
Disorders as a Possible Cause,” Journal of Women’s Health & Gender-Based Medicine 11 (2002): 39–44. 4. Vanessa R. Byams et al., “Evaluation of Bleeding Disorders in Women with Menorrhagia: A Survey of Obstetrician-Gynecologists.” American Journal of Obstetrics and Gynecology 207, no. 4 (2012): 269.e1–e5. 5. Judith Baker et al., “US Hemophilia Treatment Center Population Trends 1990–2010: Patient  Diagnoses, Demographics, Health Services Utilization,” Haemophilia 19 (2013): 21–26. 6. F. Rodeghiero et al., “Epidemiological Investigation of the Prevalence of von Willebrand Disease,” Blood 69 (1987): 454. 7. I. Plug et al., “Bleeding in Carriers of Hemophilia,” Blood 108, no. 1 (2006): 52–56.

Originally published in PEN, August 2017