Blood

Hemophilia: From Vitalism to Vampires

Halloween season is here! I’m already seeing decorations going up of ghosts, ghouls and… vampires. Vampires are steeped in horror lore, because they thrive on human blood. And blood has fascinated humankind throughout its history. I thought I’d run a post from almost ten years ago, about vampires, blood and hemophilia.

Dracula’s next victim!

Blood at once attracts and horrifies; it is the stuff of legends and tales, myths and medicine. I recently read the classic Dracula [read the book review below] and was amused to read how Dr. Van Helsing wanted to help the young Lucy, a victim of a vampire, by giving her a transfusion of blood. “Is it you or me?” he asks Dr. John Steward, about which one will roll up their sleeve to donate. Steward replies, “I am younger and stronger, Professor. It must be me.”

Steward offered his blood based on the concept of vitalism, that blood contains the traits of the being in which it flowed—a concept that was unchallenged for 1,500 years. Later in the book, Van Helsing says to Lucy’s fiancé Arthur, “John was to give his blood, as he is the more young and strong than me…. But now you are here, you are more good than us, old or young, who toil much in the world of thought.
Our nerves are not so calm and our blood so bright than yours!”

So Arthur becomes the better blood donor because he is calm and not scholarly! Of course, this is nonsense, but author Bram Stoker fell for the widespread belief in vitalism when he wrote his book. Dracula isn’t so picky; he pretty much would drink anyone’s blood.

Douglas Starr tells us in his book Blood* that the Egyptians saw blood as the carrier of the vital human spirit, and would bathe in it to restore themselves. Roman gladiators were said to have drunk the blood of their opponents to ingest their strength. “Our own culture attaches great value to blood, with the blood of Christ as among the holiest sacraments, blood libel as the most insidious slander, the blood-drinking vampire as the most odious demon.”*

Vampires… which are repelled by garlic and crucifixes (the two seemingly have nothing to do with one another). Yet rather than secure eternal spiritual life by consuming wine that has been transformed into Christ’s blood during Christian mass, Dracula drinks human blood to extend his physical life. 

The only thing scarier than vampires is the proliferation of teen movies about vampires!

*Starr, Douglas (2012-09-05). Blood: An Epic History of Medicine and Commerce (Kindle Locations
97-101). Knopf Doubleday Publishing Group.

Great Book I Just Read

Dracula  [Kindle]

By Bram Stoker

This classic, wonderful and visionary, has inspired an entire genre of books and movies. Jonathan Harker, a young English lawyer, is summoned to Castle Dracula in Transylvania to finalize a real estate transaction with the eerie Count Dracula, who is purchasing property in London. Harker is warned by local peasants, who give him crucifixes and other charms against evil. As a guest, Harker soon notices strange things: the Count has no reflection, is never present in daylight, and scales the castle walls downward, like a lizard. Unable to escape, Harker is soon a prisoner, until the Count reaches London, with 50 boxes of earth. The novel is told only through letters and diary entries of the main characters, including: Harker’s fiancée, Mina Murray; her friend Lucy Westenra, who is bitten by Dracula and slowly turns into a vampire; Dr. John Seward, Lucy’s doctor and once beau. Harker reappears in Budapest and eventually returns to London. Dr. Van Helsing, an expert on vampires, is called in from Holland to help save Lucy. Everyone realizes Dracula’s scheme to populate
London with the “Undead”—vampires. When Mina is bitten, and begins to turn into a vampire, the men sterilize the boxes of earth, set about London. Dracula, having no haven to stay when dawn comes, flees back to Transylvania, while the men pursue him. This is a fantastic story, though the language is not lofty or even that clever, with memorable characters, and cleverly told in letters and diaries. Perfect October reading. 5/5 stars.

The Bloody Book of Blood

Like the pages out of Ray Bradbury’s Fahrenheit 451, we’re seeing books get banned in some states. Math books, even!  Our favorite hemophilia archivist, Richard Atwood of North Carolina writes, “School libraries, and even their school librarians (can you believe it?), are under attack, with more and more books being banned for their content.” Richard provides a review below of juvenile literature that explains blood, including hemophilia, to young readers. He adds, “Kids seem to enjoy being grossed out!” And hopes it does not get banned!

This text on blood in Kelly Regan Barnhill’s  2010 book, The Bloody Book of Blood, is classified as juvenile literature. Hemophilia is included among the numerous blood topics. Hemophilia is explained in the section “Blood Disorders” that is accompanied with a photograph of a bruise on page 22. The text states: “Hemophilia is a rare, but serious blood disorder. This disease keeps a person’s blood from clotting properly. Patients with hemophilia need to be extra careful. A cut can bleed for days, and it may never heal completely. Even a small bruise can be a big problem. The extra blood pools under the skin, causing the bruise to grow to a huge size.”

Hemophilia is defined in the glossary, along with a pronunciation guide. The text states: “hemophilia (hee-muh-FIL-ee-uh) — a health condition in which blood does not clot normally.”

This is an educational book, part of The Amazingly Gross Human Body series that includes texts on blood, vomit, spit, snot, sweat, and… pee. This short book with a large font includes one page for a glossary, one page for an index, one page for “read more” and internet sites, 16 illustrations, and 4 “gross facts.” Michael Bentley, a professor of biology at Minnesota State University, Mankato, acted as a consultant.

“The author simplified the definition of hemophilia appropriately for juvenile readers, yet a simple note on proper treatment, and maybe genetics, would be beneficial,” Richard adds.

Kelly Regan Barnhill.  2010. The Bloody Book of Blood. Mankato. MN: Capstone Press. 32 pages.

Texts That Protect

If something big were happening, you’d want to know about it right away, especially if it concerned the factor you or your loved one uses. The Patient Notification System (PNS) is a free service that will notify you about any withdrawals, recalls or warnings concerning your specific product, and even ancillaries. Are you signed up with it?

Launched in 1998 by the Plasma Protein Therapeutics Association (PPTA), a group that is supported by manufacturers of plasma-based products and recombinant products, this system has diligently sent out hard copy, telephone and email notices of any changes in your prescription drugs.

There was a time in the early 2000s when these were frequent, as there were shortages, plant shut-downs, recalls, products taken off line, and more. You can imagine that the specter of the mass HIV infections in the blood supply in the 1980s led to this unique and vital service.

There’s good news now: stringent manufacturing practices, better donor screening and genetically-engineered products means that notices of safety concerns are almost a thing of the past.

Are you signed up? You should be. It’s free, confidential, and now fast! Just recently, the PNS added notifications by text, probably the fastest route these days.  You will want to be up to date on any changes in the product you use.

Sign up for the PNS at www.patientnotificationsystem.org or call 888-UPDATE-U.

Plasma Awareness Week

This week marks the first ever International Plasma Awareness Week, an opportunity to recognzie the importance of plasma in our lives, and the need to donate blood to provide the life-saving liquid to those in need. It’s especially important to many with hemophilia throughout the world as donated blood donations can be fractionated into factor VIII and IX for many hemophilia patients who use plasma therapy.
You learned this in high school science class but here it is again: Plasma is the clear, straw-colored liquid portion of blood that remains after red blood cells, white blood cells, platelets and other cellular components are removed. It is the single largest component of human blood, comprising about 55 percent, and contains water, salts, enzymes, antibodies and other proteins. It is a clear, straw colored liquid that is 90% water and serves as a transporting medium for cells and a variety of substances vital to the human body.
In the US, we often think only of recombinant factor when we think about treating hemophilia, but plasma therapies remain the mainstay for many with hemophilia, those with inhibitors and undergoing ITI, and those with von Willebrand disease patients.
In addition, plasma protein therapies are used to in emergency and surgical medicine.Plasma protein therapies are not interchangeable and have been defined by regulators as sole-source biologic products because no generics or substitutions exist. In addition, their biological nature demands storage and handling requirements by specialty distributors that ensure their safety.
Source plasma is plasma that is collected exclusively for further manufacturing through a process called plasmapherisis. Recovered plasma is collected through whole blood donation which has been separated into its cellular components.
Safety and quality of plasma protein therapies is the top priority of the plasma protein therapeutics industry. Both collectors and manufacturers adhere to strict regulatory policies and have instituted Good Manufacturing Practices in every step of plasma collection and manufacturing processes.

Plasma-derived therapies depend on the generosity and commitment of healthy donors. Source plasma is collected in over 450 specialized donor centers in the U.S., Canada, Germany, Austria and the Czech Republic. Source plasma collection in the U.S. is regulated by the Food and Drug Administration and by the European Medicines Agency and national regulatory authorities in Europe. Additionally, 436 plasma collection centers are also certified by the International Quality Plasma Program (IQPP), a rigorous, voluntary program that goes beyond regulatory requirements to help ensure donor safety and further improve the quality of plasma used to manufacture therapies.
This is a good week to donate blood then! If you can make the time, please visit your local blood donation center and give the gift of life.
See www.donatingplasma.org and www.pptaglobal.org  for more information.

International Plasma Awareness Week is sponsored by the Plasma Protein Therapeutics Association and its member companies.

The Bloody Ban


According to the CDC, 4.9 million Americans require a transfusion of blood or blood products daily. Is our blood supply safe enough to infuse into them?

That’s the debate raging right now. At issue? Whether gay men can be allowed to donate blood. The FDA has a policy that men who have had sex with another man (MSM), even once, since 1977 are deferred indefinitely from donating blood. On June 11, an advisory committee to the Department of Health and Human Services, composed of health experts from around the country, voted 9-6 to maintain that bans. Is this fair, unfair, discriminatory, accurate?

Mark Skinner, president of the World Federation of Hemophilia, and a lawyer, believes the ban should be upheld. He writes in an article in US News & World Report (posted July 26, 2010), “Recent CDC data indicate that while U.S. HIV infection rates are falling in heterosexuals and intravenous drug users, they are rising among men who have sex with other men and are 44 times that of other men. Additionally, other pathogens that might also be transmitted through high-risk sexual behavior are not fully understood.” Mark stresses that the safety of a recipient of a blood donation comes first. Looking at the scientific data only, the rise in HIV rates and their pattern, the ban should stay for now.

Alternatively, some believe the ban is unfair, needless and discriminatory. Representative Mike Quigley, Democrat from Illinois who sits on the House Committee on Oversight and Government Reform, voices this concern in a counter piece to Mark’s. The ban was put into place during the HIV contamination of the nation’s blood supply in the 1980s. Yet, a man who has had sex with an HIV-positive woman can donate after waiting a year. Fair? Quigley is concerned that there is not enough blood to be donated, and that the gay community represents a willing source of blood donations. Quigley wants a way to separate the high risk gay donors from the low risk, so that at least some in the community can participate.

Not good enough for Mark Skinner, who puts the focus on the recipient’s safety. But he bridges the debate by adding, “While many perceive a disconnect between leading gay rights and end-user patient organizations, these groups have more in common than recent discourse reflects. Both have been disproportionately impacted by the HIV epidemic and for most of the past quarter-century have worked toward shared goals including preventing the virus’s spread. Both share a strong commitment to a safe national blood supply.”

There’s no right or wrong answer at this point, but you can read more about this by Googling “blood donor ban.” If anything, it’s a great exercise in tolerance, science, altruism and politics. I wonder if anyone has interviewed the recipients about how they feel?

Great Book I Just Read
Undaunted Courage by Stphen E. Ambrose

Christopher Columbus may have put America on the map, but Meriweather Lewis put a map to America. One of the great adventure stories of all time, the amount of courage needed by this 30 year old, setting out to discover what lay beyond the Mississippi River, was immense. A close confident of Thomas Jefferson, who had unbridled expansionist plans, he set out in 1803 to find the fabled water passage to the Pacific Ocean. There was none, but Lewis (and Clark!) had an adventure like no other. From Philadelphia to St. Louis through Idaho and eventually Oregon in two years, they charted and mapped the US under the most primitive of circumstances, and discovered new species of plants and wildlife. They met and negotiated with the Indians, the most terrifying of which were the Sioux. A great study in leadership and courage, it has a sad outcome for Lewis in the years following his celebrity status. An amazing American hero. Four stars.

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