The Sacred Pint

The sacred pint alone can unbind the tongue. James Joyce

Following up on my blog of June 30, here’s more fascinating facts about blood. This time, what contribution war made to blood transfusion services. In Nine Pints: A Journey Through the Money, Medicine, and Mysteries of Blood , Rose George shares, in reference to World War II, how “London’s authorities had spent years readying for calamity on all fronts. But they had not thought it necessary to prepare any blood.” Below are excerpts from her excellent book!

  • In 1937, the English secretary of state for war had been asked what the nation proposed to do about a mass blood supply. He said, “It is more satisfactory to keep our stores of blood on the hoof.” He meant that the best way to store blood was how nature intended: inside a human body.
  • By World War II, blood transfusion had been fully accepted by the British military, which planned in prewar years to set up an efficient blood supply to its forces and operated it extremely effectively. Field Transfusion Unit trucks carried refrigerators containing 1,100 pints of fresh whole blood. [This had a very short life span!]
  • The actual emergency blood supply for London, a city of several million people, consisted of the stock kept by four London county hospitals for urgent maternity use: eight pints.
  • Yet in wartime Barcelona, Frederic Durán-Jordà, a Catalan physician, had successfully pioneered the mass collection, storage, and delivery of blood. He made blood mobile, transporting it to the front line in glass bottles in a converted fish van. Blood was withdrawn, mixed with citrate solution, then bottled. Barcelona’s blood was good for eighteen days.
  • We owe a lot to Percy Oliver: Oliver’s was the world’s first voluntary blood panel and the beginning of a shift to a model of altruistic blood donation in Britain that has endured one hundred years.
  • Dame Janet Maria Vaughan was a British physiologist, academic, and academic administrator. She researched in hematology and radiation pathology. In early April 1939, during World War II, she gathered some medical colleagues in her Bloomsbury flat; they would change modern medicine. She initiated creation of national blood banks in London, setting one up with Frederic Durán-Jordà. She used a modified milk bottle for blood collection and storage.
  • She used ice-cream vans full of her bottled blood through bombed and blacked-out streets. Most of the drivers were women.

• The war embedded the idea of blood donation in popular consciousness like nothing else had done.

• For British Major General W. H. Ogilvie, “the greatest surgical advance of this war, more important even than penicillin, is the development of the transfusion service.”

A departure from the topic of hemophilia, we are proud to promote…

This week we are taking a pause on the topic of bleeding disorders to share with you a very exciting project one of our team members has created!

Our editor of over 20 years, Sara Evangelos, has co-authored a young adult book titled Somewhere Besides Denver. We are so excited for Sara and this new book. Please read the synopsis below and check it out for yourself by visiting https://somewherebesidesdenver.com/

Denver, 1907: Three spirited teenage girls leave their homes on the prairie for a yearlong European Grand Tour. Violet and Marion, members of Denver’s social elite, yearn for more than society parties and safe marriages. Helen, a rancher’s daughter whose mother has abandoned the family, resents being an outsider. All three girls long to stretch the limits of what’s “suitable” for young women.

In Paris, the teens escape their chaperone and explore the city’s seamy side. Marion falls hopelessly in love with her drawing tutor, who has a history of breaking pupils’ hearts. Violet meets a chic clothing designer who encourages her to follow a career in fashion—and to ditch the corsets. In London, during the 1908 Olympics, Helen conducts a secret bicycle search for her long-lost mother, and the girls join the growing suffrage movement, barely escaping a melee at a women’s rights demonstration.

During their travels, the teens ride the Twentieth Century train and sail on the Lusitania. They meet a radical suffragette, two nosy gossips, a ground-breaking African artist, a fascinating photographer, and some fancy-dressed dogs in jeweled outfits. By the time they sail home to America, Marion, Violet, and Helen have found the confidence they need, at a time when women craved freedom—but men held the power. They decide to rebel against society’s rules, and to follow their own paths as women of the new century.

Visit https://somewherebesidesdenver.com/ for more information!

Give a Pint!

I love reading about medical history, and naturally blood is a fascinating topic. Rose George’s book Nine Pints: A Journey Through the Money, Medicine, and Mysteries of Blood is snappy and witty, and full of facts. I loved all the statistics, interwoven with real life stories of individuals. I appreciate the focus on the suffering and plight of those with hemophilia, who contracted HIV and hepatitis C. I know some of the people she quoted. George has highlighted some very important and crucial concerns: plasma donation practices, cultural differences regarding blood. A chapter on leeches– so interesting! As I am involved internationally in helping patients in developing countries gain access to clotting factor, I was very interested in the global situations she presented, especially India and Nepal, two countries I have been to many times. Here are some highlights from the book, which I strongly recommend reading:

• Every three seconds, somewhere in the world, a person receives a stranger’s blood. Globally, 13,282 centers in 176 countries collect 110 million donations.

• The mythical Gorgon Medusa, with her head of snakes, showed the two-faced nature of blood best: the veins on her left side contained blood that was lethal, while the right side gave life. Transfusions can be two-faced, too. The right type of blood can save your life; the wrong one can kill you.

• The bone marrow produces two million red blood cells every second. Daily, the blood’s thirty trillion red cells do a full circuit of the body, traveling about twelve thousand miles.

• Forty percent of Caucasians have type A blood, but only 27 percent of Asians. 19 The fact that O-type people are more susceptible to cholera was first noticed in 1977.

• Cold War Americans thought blood type so important that they tattooed it onto adults and children.

• A nation-state needs 1 to 3 percent of its population to give blood to maintain an adequate blood supply, 36 the higher the better.

• In Luxembourg only 14 percent of people give blood; over the border in France, it’s 44 percent.

• A leech bite will usually feel like a vague sensation, not a nip or scratch. Once their teeth are engaged, they emit the best anticoagulants known to exist, so that their blood meal keeps flowing long after they have stopped feeding, often for up to ten hours. The leech is in many ways a simple animal, but its anesthetic and anticoagulant have yet to be bettered by science.

• In 1985, a three-year-old boy from Massachusetts had his ear bitten off by the family dog. He was taken to Boston’s Children’s Hospital, where his surgeons proceeded as they usually did with amputated ears. But the blood couldn’t circulate well in the tight ear area. Leeches were flown in and attached to the area. They drew blood away from the wound and kept the circulation to the newly attached ear.

• The first barber-surgeon on the registry of the Worshipful Company of Barbers was recorded in 1312. The bleeding barber is the reason modern barbers display red and white striped poles: the pole was a stick for the patient to grip; the white stripes were the bandages, the red stripes the blood.

More to come next time!!

Factor your way: empowering you to achieve your goals

Sponsored Content

This is a paid public announcement from Takeda 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 bleedingdisorders.com website. LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

Submitted by Takeda

What is hemophilia?

Hemophilia is a rare genetic bleeding disorder that prevents blood from clotting normally.1

At the time of a bleed, proteins in your blood called clotting factors form a clot and stop the bleeding.2,3 However, if you have hemophilia, your blood lacks clotting factors (such as factors VIII and IX), and as a result, you may bleed for a longer time after an injury than you would otherwise.4,5

Replacing what’s missing

The current standard of care for hemophilia is factor replacement therapy.6 Factor therapy is a proven treatment with decades of real-world use and an established safety record.7,8

Factor therapy replaces the missing blood-clotting proteins that are naturally found in your blood.9 It is administered via an intravenous injection (directly into your bloodstream), making these proteins available immediately for use.

Factor therapy can be used in different situations such as on-demand (to treat an ongoing bleed), prophylaxis (to prevent bleeds before they occur), and before or after surgery.6,10

Other treatments for hemophilia

The first non-factor therapy option more recently became available.11 To reduce bleeding, it mimics part of the function of missing clotting proteins by bridging other factors. It is administered subcutaneously and is intended for prophylactic use.

Individualizing your treatment with factor

With factor treatment, your healthcare provider (HCP) can individualize your regimen to align with your unique needs and help you achieve treatment goals.

When determining an appropriate treatment plan, your HCP looks at how factor is processed in your body; this is called pharmacokinetics (PK).13 Many factors such as body weight, age, joint status, activity levels, and bleeding frequency are used to better understand how much and how often you require factor.12

A treatment tailored to your lifestyle and activities may help make it easier for you to stick to your prophylaxis. This may help preserve your joint health by significantly reducing bleeding.12

CASE STUDY

Individualizing factor therapy can make a world of difference

Josh
Age 15 years

Josh is an active teen who enjoys playing sports. He is currently taking factor every other day (25 IU/kg) but has recently been experiencing more bleeds, particularly in his ankles. At his HCP visit, Josh mentions that he has increased his tennis practice from once weekly to 3 nights/week.

Due to an increase in Josh’s activity level, and a considerable growth spurt, he may not always be receiving optimal coverage from his current dosing regimen. His HCP recommended adjusting Josh’s dose of factor VIII and changing his infusion schedule.

After taking a few blood samples, they were able to determine what Josh’s factor levels would be at various time points following an infusion. They agreed to an individualized dosing schedule that was appropriate for Josh’s current activity level. Josh’s new dose is 35 IU/kg three times a week.

Since switching to the new dosing schedule, Josh’s bleeding has been reduced even with his increased activity.

 

 

 

 

 

 

 

 

 

Visit bleedingdisorders.com for more information about hemophilia and individualized factor therapy.

Supporting literature:

  1. Livnat T, Barg AA, Levy-Mendelovich S, Kenet G. Rare bleeding disorders—old diseases in the era of novel options for therapy. Blood Cells Mol Dis. 2017;67:63-68.
  2. Smith SA, Travers RJ, Morrissey JH. How it all starts: initiation of the clotting cascade. Crit Rev Biochem Mol Biol. 2015;50(4):326-336.
  3. Berg JM, Tymoczko JL, Stryer L. Many enzymes are activated by specific proteolytic cleavage. Biochemistry. 5th ed. New York, NY: WH Freeman; 2002. https://www.ncbi.nlm.nih.gov/books/NBK22589/. Accessed April 18, 2019.
  4. Hemophilia. National Heart, Lung, and Blood Institute (NHLBI). https://www.nhlbi.nih.gov/health-topics/hemophilia. Accessed April 17, 2019.
  5. Hemophilia. Mayo Clinic. https://www.mayoclinic.org/diseases-conditions/hemophilia/symptoms-causes/syc-20373327. Accessed April 17, 2019.
  6. Rodriguez-Merchan EC. What’s new in orthopedic surgery for people with hemophilia. Arch Bone Jt Surg. 2018;6(3):157-160.
  7. Pipe SW. New therapies for hemophilia. Hematology Am Soc Hematol Educ Program. 2016;(1):650-656.
  8. Franchini M. Current management of hemophilia B: recommendations, complications and emerging issues. Expert Rev Hematol. 2014;7(5):573-581.
  9. Rolstad EB. Perceptions of men with moderate to severe hemophilia regarding the management of their chronic disorder and utilization of community-based support. Am J Mens Health. 2015;9(6):486-495.
  10. Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388(10040):187-197.
  11. National Hemophilia Foundation. MASAC document #255. Available at: www.hemophilia.org. Accessed May 2, 2019.
  12. Valentino LA. Considerations in individualizing prophylaxis in patients with haemophilia A. Haemophilia. 2014;20(5):607-615.
  13. Pharmacokinetics. The Free Dictionary by Farlex. http://medicaldictionary.thefreedictionary.com/pharmacokinetics. Accessed April 17, 2019.
Commitment to our community is always a priority for Takeda Hematology (previously part of Shire and Baxalta). As a leader in hemophilia research, Takeda continues to innovate on your behalf, developing programs and services that support your efforts each step of the way. Takeda is focused on providing advanced hematology treatments for today and innovating for the future.

Copyright © 2019 Takeda Pharmaceutical Company Limited. All rights reserved. All trademarks are the property of their respective owners.

S48266 05/19

Patient Newsletter Article 20190226 0.6

When to Suspect an Inhibitor

Let’s face it: an inhibitor is a major complication of hemophilia. It develops when the body’s immune system does not recognize infused factor as a normal part of blood. Instead, the body thinks that factor is a foreign invader, like a virus or germ, and it develops antibodies to attack the factor and make it harmless—and useless. So despite an infusion of factor, your child continues to bleed. Prolonged bleeding, even after a factor infusion, is the most common sign that your child may have an inhibitor. But other symptoms may also tip you off.

Unresolved bleeds

You may suspect an inhibitor the hard way: when factor no
longer works well to stop bleeds. If you’re new to hemophilia, this may be
difficult to judge—how long should it
take for a bleed to stop after an infusion? It may not immediately dawn on you
that the infused factor isn’t working properly, especially if your child is
already well into a muscle or joint bleed. It’s common for parents to think
that they simply need to give their child more frequent infusions, or give a
higher dose per infusion. If you think your child’s bleed is not resolving
normally, or wonder whether you should dose higher or more frequently, please
call your hemophilia treatment center (HTC).

Increased bruising

Bruising in young children with severe hemophilia is common.
But if your child is on prophylaxis and you notice increased bruising, this may
be a sign of an inhibitor.

Routine clinic visit

A blood test at your child’s HTC comprehensive clinic visit
can identify an inhibitor. Low-level inhibitors are often diagnosed in this
way. It’s wise to have a child with hemophilia tested for inhibitors routinely.
Learning that he has an inhibitor prior to surgery or a major bleed allows
parents to have a plan in place and the correct treatment on hand.

Breakthrough bleeds while on prophylaxis

Prophylaxis is the scheduled infusion of factor to help
prevent bleeding. Many children with hemophilia on prophylaxis receive factor
two to three times a week, enough to allow circulating factor to prevent most
spontaneous bleeds and abnormal bruising. When a child on prophy starts
bruising or bleeding more often than usual, an inhibitor may be inactivating
some of the factor, lowering his factor level and increasing his risk of
bleeding.

Bleeding after surgery

Any kind of surgery on a child with hemophilia requires
careful planning and monitoring of factor levels, and any child who continues
to bleed following surgery, even with adequate factor, should be immediately
tested for an inhibitor. Ideally, all children with hemophilia should be tested
for an inhibitor before any surgery.
If you see any kind of bleeding following surgery, call your HTC immediately.

Reaction following infusion

An allergic reaction is a response by the immune system to
environmental contaminants such as pollen, animal dander, or food. It can also
happen after an infusion of factor. Symptoms may include sneezing; itching;
hives; rapid swelling of the skin, neck or face; wheezing; faintness; fast
heart rate; low blood pressure. Allergic reactions are especially worrisome
with hemophilia B. An allergic reaction after a factor IX infusion is sometimes
the first sign that an inhibitor to factor IX has developed. A whopping 45% of
people with hemophilia B and inhibitors also develop allergic reactions at
about the same time that they develop inhibitors.

Don’t downplay allergic reactions. They may start out mild but then increase in severity after repeated exposure to products containing factor IX, often to a serious, life-threatening allergic reaction called anaphylaxis. If your child has severe hemophilia B, his first 20 infusions of factor IX concentrate should be done in a hospital or clinic with expertise in treating severe allergic reactions.

Following a major bleed or emergency

Whenever your child receives large amounts of factor—in
response to a major bleed or during and after surgery—he may be at higher risk
of developing an inhibitor. Experts aren’t sure if large amounts of factor
stimulate inhibitor development, or if the body is more susceptible to
inhibitor development during a medical crisis because the immune system is on
high alert. But whatever the reasons, be aware that the risk of developing an
inhibitor is slightly higher during an illness or surgery.

Later in life

If a person with hemophilia develops an inhibitor, it’s usually while he’s a child, almost always before exposure day 100. And he probably has severe hemophilia. But in rare cases, an inhibitor develops in a teen or an adult—usually in people with mild or moderate hemophilia, and usually after intensive exposure to factor during and after surgery or traumatic injury.

Inhibitors are scary to contemplate. Even when your child
passes exposure day 100, don’t be lulled into thinking that he may never
develop one. You can always request an inhibitor test from your HTC. Never try
to diagnose on your own, or change your child’s dosing regimen on your own.

In your favor? A great array of educational resources, listed in this issue, and a wonderful team of medical experts. Don’t be afraid! Be proactive, just as you were when you learned about hemophilia. Inhibitors are one obstacle to overcome on your family’s hemophilia journey.

PEN 2.11     © 2011 LA Kelley Communications