October 2020

From starting school to starting young adult life, BleedingDisorders.com can help at every mile of the journey

Our publications address the many challenges you face as you reach new milestones in life with hemophilia. For this week’s HemaBlog, we suggest you check out this website to learn more about handling life’s many phases and changes!

Laurie

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 a Takeda website. LA Kelley Communications always advises you to be a savvy consumer when contacting any company; do not reveal identifying information against your will.

Every life has its twists and turns, and that’s even more true for young people with bleeding disorders and their caretakers. BleedingDisorders.com is right there with you through every mile of the journey, with info and resources designed to help you and your family navigate change—whether that means your child’s first day of school or their last day living at home.

As school is getting underway, get the team together
Starting school is a big step—for you and your child! To make it as safe as possible, it’s a good idea to go over some important topics with key members of your school team, including your child’s teacher, the school nurse, the guidance counselor, the gym teacher, and someone from administration.1 You play an important role in educating them about your child’s bleeding disorder and what they should expect with your child under their supervision. And of course, this meeting can be virtual or in-person, depending on your school’s situation.

Wondering what other topics you should cover with your ace team of educators? Bleedingdisorders.com has put together a helpful list.

Growing up and taking control
Young adulthood is another important transitional period for people living with a bleeding disorder. For some, it might be the first time in their life they are responsible for managing their treatment and their symptoms on their own. And whether they are moving into a college dorm or just moving out, you and your child should make sure you’re both prepared for the transition.

Here are a couple key tips for your family that could help make the move go smoother:

Prep for an Emergency
With hemophilia, a bleeding emergency can come at any time. Preparing an Emergency Care Letter and an emergency bag with everything they’ll need can save time and stress.2

Ace Your Storage Quiz
Make sure they know exactly how to store and handle their treatment. Products that have been improperly stored or have expired may not be as effective in preventing or treating bleeding episodes.3

See the full list of helpful tips for young adults at BleedingDisorders.com.

Stay connected
No matter what life stage you or your child is at, it’s important to have a good relationship with your local Hemophilia Treatment Center, where you can get expert advice about managing a bleeding disorder. Your healthcare provider team can also help with any big life changes your family may face. And of course, connect with the Bleeding Disorders community on Facebook, Instagram, YouTube, and Twitter, and sign up to get the latest news and updates.
References:
  1. Phyllis McIntosh; HemAware. Back to school. 2006. Accessed September 14, 2020. https://www.hemophiliafed.org/uploads/Back-to-School_Hemaware.pdf
  2. Emergency preparedness. Steps for Living website. Accessed September 14, 2020. https://stepsforliving.hemophilia.org/step-up/treatment/emergency-preparedness
  3. Frase M. Safely storing factor at home. HemAware website. June 21, 2010. Accessed September 14, 2020. https://hemaware.org/bleeding-disorders-z/safely-storing-factor-home

Copyright © 2020 Takeda Pharmaceutical Company Limited. Lexington, MA 02421. 1-800-828-2088. All rights reserved. TAKEDA and the TAKEDA logo are trademarks or registered trademarks of Takeda Pharmaceutical Company Limited.

US-NON-2287v1.0 09/20

Snakes and Hemophilia

Just in time for Halloween, our amazing archivist, Richard Atwood of North Carolina, has found another reference to hemophilia in an unlikely place—a novel about snakes. The Snake, by John Godey, published in 1978, is a novel about... a snake.

It’s called a black mamba, though olive-slate in color, extending 11 feet, 2 inches. The black mamba is the largest and deadliest poisonous snake in Africa. The snake arrives in New York City in a plywood box with a burlap lining and air holes.

A Greek bought the snake in Elizabethville, then lost it in a poker game in Maputo. Matt Olssen, a seaman returning from Africa to the States, wins the snake and wants to sell it to a zoo. After docking in Brooklyn, Olssen takes his snake-in-a-box and begins drinking. In Central Park late that September night during a stifling heat wave, a thug named Ramon Torres mugs, then shoots Olssen dead. The box breaks open and the snake bites Torres twice in his thigh. Torres dies that night at East Side Hospital.

The next evening Roddy Bamberger leaves his date at the outdoor Delacorte Theatre. Walking past Belvedere Lake, Bamberger steps on the snake and is bitten three times in his left thigh. Bamberger dies at East Side Hospital. With two deaths to solve, and the city in a panic, Captain Thomas Eastman of the NYPD is put in charge of finding the unidentified snake in Central Park - an area covering 840 acres, of which 150 is water. The Reverend Sanctus Milanese of the Church of Purification proclaims that the snake is Satan, or Satan’s messenger.

Captain Eastman recruits Mark Converse, a young herpetologist, to locate the snake. Jane Redpath, a graduate student, takes photographs in the menagerie at night. She is accompanied by her boyfriend, Jeff, a former football player at Columbia. Jeff is bitten on his forearm, and dies at East Side Hospital. The snake then bites Alvis Parkins, a 15-year-old boy, twice in the neck, but Alvis is run over by a car before he can reach a hospital. Fortuitously, Redpath took a photo of the snake, that can now be identified for the correct antivenin.

Later, the snake chases a rat into an apartment and bites a family from Trinidad: Webster McPeak, wife Emily, son Webster Junior, and daughter Charlene. Even with black mamba antivenin, Emily and Charlene die at West Side Hospital. On the twelfth day of the heat wave, Converse spots the snake sunning on a rock in the East Meadow, but a police helicopter scares the snake away. A follower of Reverend Milanese named Graham Black is also searching for the snake. Black is bitten on his right leg and receives the antivenin. The police fire Converse. Reverend Milanese organizes Operation Pillar of Fire to burn the snake out of Central Park. The snake escapes and bites a couple three times in the back of a taxi. Another Milanese disciple is bitten by the snake after it is beheaded.

But it’s not over yet... the snake leaves 13 eggs in her burrow in Central Park!

It’s interesting to note that on page 56 in the book, a television newscast shows the milking of a Russell’s viper’s venom at a Brazilian snake farm. The film is narrated: “Not all snake poison is malign. The venom being taken from this Russell’s viper will be used as a coagulant for persons suffering from hemophilia.”

Richard comments, “The simplest of fears can sometimes be the most terrifying. The possibility of a single snake terrorizing an entire city seems realistic. The snake bites 11 individuals, killing at least six, plus she has the opportunity to bite numerous other New Yorkers during her short stay in Central Park. The revelation that Russell’s viper venom has been used to treat hemophilia as a topical hemostyptic is correct. The novel, however, does not reveal that the product has been commercially available from Burroughs Wellcome and Company as Stypven, and from Boots as Rusven. A Russell’s viper venom dilution of 1:10,000 clots hemophilic blood in 17 seconds. The commercial product uses a 1:100,000 solution, while the venom is still active at 1:1,000,000,000.”

I’ve heard of transgenic animals that are milked to collect factor, but never milking a snake for its venom to clot blood! Since Halloween is cancelled most everywhere due to the pandemic, read a good scary book this Halloween!

Is It Safe to Donate Blood in a Pandemic?

In the U.S., the first full week of September is designated National Blood Donation Week, to promote blood donations and the need for blood, for emergences and for therapeutic drugs. I normally post something about it, but with Covid-19 taking center stage, overlooked it this year. I just read The Source magazine, from the Protein and Plasma Therapeutics Association (PPTA), and found the welcome, called "Outlook," by new president Amy Efantis, very compelling. Please have a read of this excerpt, and go to "the source" itself, to download your own copy here. Remember that some of our community members still use plasma-derived products, as do many in developing countries. Is it safe to donate blood in a pandemic?

Outlook by Amy Efantis

"... in those early days of this crisis, my committed and experienced colleagues recognized even before the scourge of Coronavirus SARS-CoV-2 was officially declared a pandemic that patients might have concerns about plasma-derived therapies (PDTs) and the safety of those therapies due to COVID-19. Our member companies’ leading pathogen safety experts came together to assure patients of the safety of PDTs. They outlined the extensive safety measures that industry follows for donor recruitment and validated pathogen removal/inactivation steps during the manufacturing process, providing assurance to patients and providers about the safety of PDTs. Our statement detailing these protocols was published on our website in several languages and has been frequently cited.

"PPTA sprang into action to make sure that plasma centers, center employees, and donors were deemed essential to perform their daily activities by authorities in the U.S. and in Europe. After appeals from PPTA, the European Centre for Disease Prevention and Control defined plasma as an essential substance of human origin and encouraged plasma centers to continue collection to ensure the continuity of production of plasma-derived therapies. PPTA also engaged in efforts to make sure that PDTs, and the essential goods needed in the collection of plasma as well as manufacturing of therapies, were considered essential to ensure the movement of goods between and among countries during the pandemic. This included appeals to authorities to make sure centers received priority for personal protective equipment and medical supplies.

"In the U.S., our efforts resulted in the President’s Coronavirus Guidelines for America noting the special responsibility of the pharmaceutical supply chain, including the important role of plasma and plasma donors. In fact, donors and staff were explicitly identified in every iteration of the federal government’s advisory list of essential critical infrastructure. But even as officials at the U.S. Department of Homeland Security and the European Commission issued guidance that deemed plasma centers, staff, and donors as essential, PPTA was called upon to engage directly with local, state, and national level authorities who were not aware of or did not understand the guidance, to ensure that centers remained operational. To assist in this effort, PPTA developed a repository of documented exemptions that plasma centers could have on hand if questioned by authorities during periods of restricted movement.

"We recognized early on that plasma donors, who are essential for addressing the clinical needs of patients for PDTs, might have concerns about their safety given anxiety surrounding the contagious nature of COVID-19. PPTA facilitated the development of a preparedness checklist for centers in the early days of the pandemic. PPTA member companies took immediate actions to accommodate the safety of donors in their centers with new protocols for increased screening, the use of personal protective equipment, social distancing, and the availability of disinfectants.

"Because of government-imposed restrictions in movement, our typical inspection paradigm was disrupted. In keeping with our historical commitment to standards, PPTA submitted a proposal to the European Medicines Agency (EMA) requesting remote good manufacturing practice (GMP) inspections and parallel inventory holds, which were agreed to by EMA’s GMP/Gross Domestic Product Good Distribution Practice Inspection Working Group. We adapted inspections in our International Quality Plasma Program to virtual models, and we advised auditors to match the U.S. Food and Drug Administration’s relaxed inventory hold period from 60 days to 45 days for purposes of PPTA’s Quality Standards of Excellence, Assurance & Leadership program. These efforts reflect our willingness to be nimble in a crisis with our abiding commitment to the highest possible standards.

"PPTA’s work with other stakeholders has meant increased attention to the urgency of plasma collection. Our partnership with the Platform for Plasma Protein Users and the European Plasma Alliance resulted in the European Commission launching a call for blood and plasma donations. It also prompted the EU Health Commissioner’s issuance of a statement recognizing for the first time the importance of plasma and its use in treating rare conditions, as well as its role in fighting COVID-19.

"In the U.S and in Europe, the patient community weighed in with local and national policymakers on issues supporting Source plasma donors and shined a bright light on the urgent need for plasma to treat rare disease patients. We are proud of our efforts during the COVID-19 crisis. PPTA has seen positive results in our responses during the pandemic. But this is not the end of the story.

"Concerns over plasma availability have now replaced initial anxiety around the safety of finished product. In March and April, plasma collections were down, understandably, compared to 2019 due to government-imposed restrictions on movement in the U.S. and Europe. But the expectation was that a recovery would occur as COVID-19 cases declined... in the U.S., as quarantine protocols were loosened, there was a resurgence of COVID-19 cases in several states, including Florida and Texas, states with a strong presence of plasma collection centers. Though a recovery continues, it is far less robust than expected. So now the real work begins.

"Just as it has been challenging to predict what will happen next in the pandemic, the same holds for the future of PDTs. But we do know this much today — donations NOW will mean better outcomes in the future. To meet patient clinical need, we encourage you to:

• Contact policymakers about the urgent need for plasma donations.

• Speak out about ending outdated regulations and call for the coexistence of the private sector with the public sector to collect more plasma.

• Use your own social media channels to spread the word about the ongoing need for plasma donations.

• Donate Plasma if you are an eligible, healthy adult — patients are counting on you!

"Let’s come together to show our commitment to patients."

The Plasma Protein Therapeutics Association (PPTA) represents the private sector manufacturers of plasma-derived and recombinant analog therapies, collectively known as plasma protein therapies and the collectors of source plasma used for fractionation.

Pain Is Not the Problem…It’s the Solution

by Corey Pierce , November PEN 2020

The bleeding disorder community has a pain problem, but it’s not the problem of too much pain. The real problem is a misunderstanding of pain and its importance for our survival. Many people with bleeding disorders have come to devalue pain, because pain is often characterized as a mysterious and uncontrollable inevitability. A fallacious concept like “spontaneous bleeding” has sowed a seed in many minds that the actions we engage in are divorced from how our bodies respond. If the pain we experience is seen as inevitable, or with clouded causes, then we’re left with no other option than to manage the best we can, or trick ourselves into thinking the pain doesn’t exist.

If given the option to never feel pain again, many people would take this deal in a heartbeat. But only later would they truly understand the horrors of living without pain. What if you took this deal? Well, rare medical conditions like congenital insensitivity to pain (CIP) suggest that you shouldn’t be too attached to your fingers and toes. And you’d better hope to never get an infection or have heart failure, because you’ll be without the best warning sign nature ever created: pain. In short, your life would end much sooner. Living without pain is not a blessing—it’s a tragic, brutal curse.

If you want to understand pain, know that it results from a set of factors. Pain doesn’t arrive out of nowhere, and it’s rarely attributable to a single cause. For example, the mild ankle pain I am experiencing as I write this is not only the result of countless past ankle bleeds, but also the result of (1) standing for two hours straight while making an elaborate meal yesterday; (2) wearing “cool” shoes with no heel drop yesterday; (3) running three days ago even though I was sore; (4) eating inflammatory foods yesterday; (5) being dehydrated today; (6) slightly twisting my ankle on a tree nut while intently looking at a mailbox on a recent run; (7) not being able to ice myself after physical activity (my roommate has needed the ice more after his recent surgery); (8) not yet knowing my mileage limit while running (running is new for me since COVID-19); (9) switching to a new running shoe (is it helping or hurting?); (10) walking a mile on uneven sand recently. And so on…

If you look critically at what contributes to pain, you’ll see that it’s possible to improve your underlying condition by changing how you navigate through this world. By examining the obvious—and subtle—contributing factors to pain, you can create a template in your mind for what you should be doing and also avoiding. In my earlier example, there are complex interactions between my physical awareness, my activity level, and my shoes that I can monitor and refine over time, as I get used to my new activity—running. This introspective, reflective process is often at odds with the objectives of using pain medications.

Sadly, many people with bleeding disorders have been offered pain medications as the only solution to their pain. Too much reliance on pain meds may be nothing more than a deception that obscures the true problem(s). While effective over the short term in tamping down pain signals (the effects), pain meds also disguise the complex set of underlying factors (the causes). If we don’t address the true causes of pain, and instead focus solely on the effects with pain medication, the condition or behaviors at the root of pain will get worse, not better.

So, is pain your enemy, or is it your collaborator? If you want to live a long and healthy life, pain can’t be reduced to your tormentor Don’t let your pain be in vain by seeking remedy only through pain medications. You can’t arrive at the underlying causes of your pain when you are literally numb to them. Our pain is not what ruins life; it is what prolongs life, but only if we listen and learn from it.

Corey Pierce is a PhD candidate in public health at Oregon State University. He is currently completing his PhD dissertation, which explores how pain attitudes are connected to the experience of pain and use of pain medication. Corey has severe hemophilia A. He is active in the bleeding disorder community as an advocate and teacher of yoga, and he serves on the board of Pacific Northwest Bleeding Disorders.

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