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 The reference is an undated, unpublished manuscript by A.J. Lorenz.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!

Factor replacement–treatment you can rely on

I travel to many countries where factor is not available to patients with hemophilia and it’s heartbreaking to see the damage bleeding can do. We are fortunate in this country to have factor replacement therapy. Read below for a quick summary of its benefits, and why it’s so important for patients.

This is a paid public announcement from Shire and does not constitute an endorsement of products or services.  When you click on the links in this blog entry, you will be directed to the Shire website.  LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

When it comes to living with your hemophilia, having confidence in your treatment is important. For more than 50 years, factor replacement has been the standard of care in the treatment of hemophilia A and B with proven efficacy in preventing bleeds through prophylaxis and low risk of thrombotic events.1-4  

Factor-based therapy replaces what’s missing in the coagulation cascade for people with hemophilia, thus working within the body’s natural hemostatic process.3,5

Factor replacement represents a comprehensive approach to treating hemophilia. It can be used in multiple scenarios to meet different treatment needs that vary from person to person such as prophylaxis (routine infusion of factor to prevent bleeds), on-demand use (infusion of factor to control bleeding episodes), and during or after surgery.3,6

A one-size-fits-all treatment approach does not meet the unique needs of each individual.7 Your healthcare provider can individualize your regimen by adjusting your infusion dose and frequency (how often factor is infused) based on:

  • How your body uses factor
  • Your activity level
  • Your lifestyle needs

Individualizing your prophylactic treatment can help reduce bleed rates in some patients with hemophilia A.8

Reducing bleeds is necessary to preserve your joints. Even a single bleed matters, and there is evidence that the number of joint bleeds a patient experiences can lead to the development of joint disease.9 Remember, if bleeds are not treated immediately, this can result in permanent joint damage.2

Decades of clinical studies and real-world use have shown that early prophylaxis with factor-based treatment can reduce the risk of joint bleeds and preserve joint health in some patients with hemophilia A.2,10-15

To effectively manage your hemophilia, be sure to ask your healthcare provider about an established treatment that offers options for individualization. With decades of documented success in managing, controlling, and reducing bleeds in more than 170 clinical studies, factor treatment remains a trusted and well-studied treatment option in patients with hemophilia.1, 15, 16

Commitment to the hemophilia community is always a priority to Shire. As a leader in hemophilia research, Shire continues to innovate on your behalf, developing programs and services that support your efforts each step of the way. Shire is focused on providing advanced hematology treatments for today and innovating for the future. 17

Talk to your healthcare provider to learn more about how factor works and all the ways it can help patients like you with hemophilia. Visit www.bleedingdisorders.com to discover if factor treatment is right for you.

References

  1. Center for Biologics Evaluation and Research. User fee billable biologic products and potencies approved under Section 351 of the PHS Act. http://www.fda.gov/AboutFDA/CentersOffices/OfficeofMedicalProductsandTobacco/CBER/ucm122936.htm. Accessed July 17, 2018.
  2. Price VE, Hawes SA, Chan AKC. A practical approach to hemophilia care in children. Paediatri Child Health. 2007;12(5):381-383.
  3. Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388:187-197.
  4. Coppola A, Franchini M, Makris M, Santagostino E, Di Minno G, Mannucci PM. Thrombotic adverse events to coagulation factor concentrates for treatment of patients with haemophilia and von Willebrand disease: a systematic review of prospective studies. 2012;18:e173-e187.
  5. Berg JM, Tymoczko JL, Stryer L. Many enzymes are activated by specific proteolytic cleavage. In: Biochemistry. 5th ed. New York, NY: WH Freeman; 2002. https://www.ncbi.nlm.nih.gov/books/NBK22589/?rendertype. Accessed July 24, 2018.
  6. World Federation of Hemophilia. Guidelines for the management of hemophilia. 2nd ed. Montreal, Quebec: World Federation of Hemophilia; 2012:1-80.
  7. Valentino LA. Considerations in individualizing prophylaxis in patients with haemophilia A. Haemophilia. 2014;20:607-615.
  8. Yamasaki N, Fujii T, Fujii T. Individualized prophylaxis can contribute to decreasing annualized bleeding rate (ABR) in adult persons with hemophilia A. 2018;24(suppl 5):1-194.
  9. Collins PW, Blanchette VS, Fischer K, et al. Break-through bleeding in relation to predicted factor VIII levels in patients receiving prophylactic treatment for severe hemophilia A. J Thromb Haemost. 2009;7:413-420.
  10. Bertamino M, Riccardi F, Banov L, et al. Hemophilia care in the pediatric age. J Clin Med. 2017;6(54):1-13.
  11. Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med. 2007;357(6):535-544.
  12. Manco-Johnson MJ, Soucie JM, Gill JC for the Joint Outcomes Committee of the Universal Data Collection, US Hemophilia Treatment Center Network. Prophylaxis usage, bleeding rates,and joint outcomes of hemophilia, 1999 to 2010: a surveillance project. 2017;129(17):2368-2374.
  13. Khawaji M, Astermark J, Von Mackensen S, Akesson K, Berntorp E. Bone density and heath-reated quality of life in adut patients with severe hemophilia. Haemophilia. 2011;17:304-311.
  14. Rodriguez-Merchan EC. Aspects of current management: orthopaedic surgery in haemophilia. 2012;18:8-16.
  15. Franchini M, Mannucci PM. The history of hemophilia. Semin Thromb Hemost. 2014;40:571-
  16. NIH Clinical Trials Registry. Ongoing and complete clinical trials using factor in patients with hemophilia. https://clinicaltrials.gov/ct2/results/details?term=Factor+VIII&recr=Closed&fund=2. Accessed July 17, 2018.
  17. Shire website. https://www.shire.com/who-we-are/areas-of-focus/hematology

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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/

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